Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3
Identifieur interne : 000C40 ( Istex/Corpus ); précédent : 000C39; suivant : 000C41Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3
Auteurs : Jonas Saute ; Andrew Da ; Alexandre Muller ; Gisele Hansel ; Alexandre De ; Fábio Maeda ; Leonardo Vedolin ; Maria Saraiva ; Diogo Souza ; Javier Arpa ; Ignacio Torres ; Luis Portela ; Laura JardimSource :
- Movement Disorders [ 0885-3185 ] ; 2011-03.
English descriptors
- KwdEn :
Abstract
Spinocerebellar ataxias (SCAs) constitute a group of autosomal dominant neurodegenerative disorders with no current treatment. The insulin/insulin‐like growth factor 1 (IGF‐1) system (IIS) has been shown to play a role in the neurological dysfunction of SCAs and other polyglutamine disorders. We aimed to study the biomarker profile of serum IIS components in SCA3. We performed a case–control study with 46 SCA3 patients and 42 healthy individuals evaluating the peripheral IIS profile (insulin, IGF‐1, IGFBP1 and 3) and the correlation with clinical, molecular, and neuroimaging findings. SCA3 patients presented lower insulin and IGFBP3 levels and higher insulin sensitivity (HOMA2), free IGF‐I, and IGFBP1 levels when compared with controls. IGFBP‐1 levels were directly associated with CAG expanded repeat length; IGF‐1 was associated with the volumetries of specific brainstem regions on magnetic resonance imaging (MRI). Insulin levels and sensitivity were related to age at onset of symptoms. Our findings indicate an involvement of IIS components in SCA3 neurobiology and IGFBP‐1 as a potential biomarker of the disease. © 2010 Movement Disorder Society
Url:
DOI: 10.1002/mds.23428
Links to Exploration step
ISTEX:6E46E47B73A2D590EF06FE38F46F2D686EDA1122Le document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3</title>
<author><name sortKey="Saute, Jonas Alex Morales" sort="Saute, Jonas Alex Morales" uniqKey="Saute J" first="Jonas" last="Saute">Jonas Saute</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Neurology Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Da Silva, Andrew Chaves Feitosa" sort="Da Silva, Andrew Chaves Feitosa" uniqKey="Da Silva A" first="Andrew" last="Da">Andrew Da</name>
<affiliation><mods:affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Muller, Alexandre Pastoris" sort="Muller, Alexandre Pastoris" uniqKey="Muller A" first="Alexandre" last="Muller">Alexandre Muller</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Hansel, Gisele" sort="Hansel, Gisele" uniqKey="Hansel G" first="Gisele" last="Hansel">Gisele Hansel</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="De Mello, Alexandre Silva" sort="De Mello, Alexandre Silva" uniqKey="De Mello A" first="Alexandre" last="De">Alexandre De</name>
<affiliation><mods:affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Maeda, Fabio" sort="Maeda, Fabio" uniqKey="Maeda F" first="Fábio" last="Maeda">Fábio Maeda</name>
<affiliation><mods:affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Vedolin, Leonardo" sort="Vedolin, Leonardo" uniqKey="Vedolin L" first="Leonardo" last="Vedolin">Leonardo Vedolin</name>
<affiliation><mods:affiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Saraiva Ereira, Maria Luiza" sort="Saraiva Ereira, Maria Luiza" uniqKey="Saraiva Ereira M" first="Maria" last="Saraiva">Maria Saraiva</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Souza, Diogo Onofre" sort="Souza, Diogo Onofre" uniqKey="Souza D" first="Diogo" last="Souza">Diogo Souza</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Arpa, Javier" sort="Arpa, Javier" uniqKey="Arpa J" first="Javier" last="Arpa">Javier Arpa</name>
<affiliation><mods:affiliation>Neurology Service, Hospital Universitario “La Paz” (HULP), Madrid, Spain</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Torres Leman, Ignacio" sort="Torres Leman, Ignacio" uniqKey="Torres Leman I" first="Ignacio" last="Torres">Ignacio Torres</name>
<affiliation><mods:affiliation>Laboratory of Cellular and Molecular Neuroendocrinology, Instituto Cajal, Consejo Superior de Investigaciones Cientificas (CSIC), Madrid, Spain</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>CIBERNED, Madrid, Spain</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Portela, Luis Valmor Cruz" sort="Portela, Luis Valmor Cruz" uniqKey="Portela L" first="Luis" last="Portela">Luis Portela</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Jardim, Laura Bannach" sort="Jardim, Laura Bannach" uniqKey="Jardim L" first="Laura" last="Jardim">Laura Jardim</name>
<affiliation><mods:affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Department of Internal Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">ISTEX</idno>
<idno type="RBID">ISTEX:6E46E47B73A2D590EF06FE38F46F2D686EDA1122</idno>
<date when="2011" year="2011">2011</date>
<idno type="doi">10.1002/mds.23428</idno>
<idno type="url">https://api.istex.fr/document/6E46E47B73A2D590EF06FE38F46F2D686EDA1122/fulltext/pdf</idno>
<idno type="wicri:Area/Istex/Corpus">000C40</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3</title>
<author><name sortKey="Saute, Jonas Alex Morales" sort="Saute, Jonas Alex Morales" uniqKey="Saute J" first="Jonas" last="Saute">Jonas Saute</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Neurology Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Da Silva, Andrew Chaves Feitosa" sort="Da Silva, Andrew Chaves Feitosa" uniqKey="Da Silva A" first="Andrew" last="Da">Andrew Da</name>
<affiliation><mods:affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Muller, Alexandre Pastoris" sort="Muller, Alexandre Pastoris" uniqKey="Muller A" first="Alexandre" last="Muller">Alexandre Muller</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Hansel, Gisele" sort="Hansel, Gisele" uniqKey="Hansel G" first="Gisele" last="Hansel">Gisele Hansel</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="De Mello, Alexandre Silva" sort="De Mello, Alexandre Silva" uniqKey="De Mello A" first="Alexandre" last="De">Alexandre De</name>
<affiliation><mods:affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Maeda, Fabio" sort="Maeda, Fabio" uniqKey="Maeda F" first="Fábio" last="Maeda">Fábio Maeda</name>
<affiliation><mods:affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Vedolin, Leonardo" sort="Vedolin, Leonardo" uniqKey="Vedolin L" first="Leonardo" last="Vedolin">Leonardo Vedolin</name>
<affiliation><mods:affiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Saraiva Ereira, Maria Luiza" sort="Saraiva Ereira, Maria Luiza" uniqKey="Saraiva Ereira M" first="Maria" last="Saraiva">Maria Saraiva</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Souza, Diogo Onofre" sort="Souza, Diogo Onofre" uniqKey="Souza D" first="Diogo" last="Souza">Diogo Souza</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Arpa, Javier" sort="Arpa, Javier" uniqKey="Arpa J" first="Javier" last="Arpa">Javier Arpa</name>
<affiliation><mods:affiliation>Neurology Service, Hospital Universitario “La Paz” (HULP), Madrid, Spain</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Torres Leman, Ignacio" sort="Torres Leman, Ignacio" uniqKey="Torres Leman I" first="Ignacio" last="Torres">Ignacio Torres</name>
<affiliation><mods:affiliation>Laboratory of Cellular and Molecular Neuroendocrinology, Instituto Cajal, Consejo Superior de Investigaciones Cientificas (CSIC), Madrid, Spain</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>CIBERNED, Madrid, Spain</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Portela, Luis Valmor Cruz" sort="Portela, Luis Valmor Cruz" uniqKey="Portela L" first="Luis" last="Portela">Luis Portela</name>
<affiliation><mods:affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
<author><name sortKey="Jardim, Laura Bannach" sort="Jardim, Laura Bannach" uniqKey="Jardim L" first="Laura" last="Jardim">Laura Jardim</name>
<affiliation><mods:affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
<affiliation><mods:affiliation>Department of Internal Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</mods:affiliation>
</affiliation>
</author>
</analytic>
<monogr></monogr>
<series><title level="j">Movement Disorders</title>
<title level="j" type="abbrev">Mov. Disord.</title>
<idno type="ISSN">0885-3185</idno>
<idno type="eISSN">1531-8257</idno>
<imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<pubPlace>Hoboken</pubPlace>
<date type="published" when="2011-03">2011-03</date>
<biblScope unit="volume">26</biblScope>
<biblScope unit="issue">4</biblScope>
<biblScope unit="page" from="731">731</biblScope>
<biblScope unit="page" to="735">735</biblScope>
</imprint>
<idno type="ISSN">0885-3185</idno>
</series>
<idno type="istex">6E46E47B73A2D590EF06FE38F46F2D686EDA1122</idno>
<idno type="DOI">10.1002/mds.23428</idno>
<idno type="ArticleID">MDS23428</idno>
</biblStruct>
</sourceDesc>
<seriesStmt><idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>IGFBP</term>
<term>IGF‐1</term>
<term>Machado‐Joseph disease</term>
<term>insulin</term>
<term>polyglutamine disorders</term>
<term>spinocerebellar ataxia</term>
</keywords>
</textClass>
<langUsage><language ident="en">en</language>
</langUsage>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Spinocerebellar ataxias (SCAs) constitute a group of autosomal dominant neurodegenerative disorders with no current treatment. The insulin/insulin‐like growth factor 1 (IGF‐1) system (IIS) has been shown to play a role in the neurological dysfunction of SCAs and other polyglutamine disorders. We aimed to study the biomarker profile of serum IIS components in SCA3. We performed a case–control study with 46 SCA3 patients and 42 healthy individuals evaluating the peripheral IIS profile (insulin, IGF‐1, IGFBP1 and 3) and the correlation with clinical, molecular, and neuroimaging findings. SCA3 patients presented lower insulin and IGFBP3 levels and higher insulin sensitivity (HOMA2), free IGF‐I, and IGFBP1 levels when compared with controls. IGFBP‐1 levels were directly associated with CAG expanded repeat length; IGF‐1 was associated with the volumetries of specific brainstem regions on magnetic resonance imaging (MRI). Insulin levels and sensitivity were related to age at onset of symptoms. Our findings indicate an involvement of IIS components in SCA3 neurobiology and IGFBP‐1 as a potential biomarker of the disease. © 2010 Movement Disorder Society</div>
</front>
</TEI>
<istex><corpusName>wiley</corpusName>
<author><json:item><name>Jonas Alex Morales Saute MD</name>
<affiliations><json:string>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
<json:string>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
<json:string>Neurology Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Andrew Chaves Feitosa da Silva MD</name>
<affiliations><json:string>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Alexandre Pastoris Muller MSc</name>
<affiliations><json:string>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Gisele Hansel MSc</name>
<affiliations><json:string>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Alexandre Silva de Mello MSc</name>
<affiliations><json:string>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Fábio Maeda PhD</name>
<affiliations><json:string>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
<json:string>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Leonardo Vedolin MD, PhD</name>
<affiliations><json:string>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Maria Luiza Saraiva‐Pereira PhD</name>
<affiliations><json:string>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
<json:string>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Diogo Onofre Souza MD, PhD</name>
<affiliations><json:string>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Javier Arpa MD</name>
<affiliations><json:string>Neurology Service, Hospital Universitario “La Paz” (HULP), Madrid, Spain</json:string>
</affiliations>
</json:item>
<json:item><name>Ignacio Torres‐Aleman PhD</name>
<affiliations><json:string>Laboratory of Cellular and Molecular Neuroendocrinology, Instituto Cajal, Consejo Superior de Investigaciones Cientificas (CSIC), Madrid, Spain</json:string>
<json:string>CIBERNED, Madrid, Spain</json:string>
</affiliations>
</json:item>
<json:item><name>Luis Valmor Cruz Portela PhD</name>
<affiliations><json:string>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
<json:item><name>Laura Bannach Jardim MD, PhD</name>
<affiliations><json:string>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
<json:string>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</json:string>
<json:string>Department of Internal Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</json:string>
</affiliations>
</json:item>
</author>
<subject><json:item><lang><json:string>eng</json:string>
</lang>
<value>spinocerebellar ataxia</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>polyglutamine disorders</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>IGF‐1</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>insulin</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>IGFBP</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>Machado‐Joseph disease</value>
</json:item>
</subject>
<language><json:string>eng</json:string>
</language>
<abstract>Spinocerebellar ataxias (SCAs) constitute a group of autosomal dominant neurodegenerative disorders with no current treatment. The insulin/insulin‐like growth factor 1 (IGF‐1) system (IIS) has been shown to play a role in the neurological dysfunction of SCAs and other polyglutamine disorders. We aimed to study the biomarker profile of serum IIS components in SCA3. We performed a case–control study with 46 SCA3 patients and 42 healthy individuals evaluating the peripheral IIS profile (insulin, IGF‐1, IGFBP1 and 3) and the correlation with clinical, molecular, and neuroimaging findings. SCA3 patients presented lower insulin and IGFBP3 levels and higher insulin sensitivity (HOMA2), free IGF‐I, and IGFBP1 levels when compared with controls. IGFBP‐1 levels were directly associated with CAG expanded repeat length; IGF‐1 was associated with the volumetries of specific brainstem regions on magnetic resonance imaging (MRI). Insulin levels and sensitivity were related to age at onset of symptoms. Our findings indicate an involvement of IIS components in SCA3 neurobiology and IGFBP‐1 as a potential biomarker of the disease. © 2010 Movement Disorder Society</abstract>
<qualityIndicators><score>7.028</score>
<pdfVersion>1.3</pdfVersion>
<pdfPageSize>612 x 810 pts</pdfPageSize>
<refBibsNative>true</refBibsNative>
<keywordCount>6</keywordCount>
<abstractCharCount>1163</abstractCharCount>
<pdfWordCount>27035</pdfWordCount>
<pdfCharCount>175924</pdfCharCount>
<pdfPageCount>43</pdfPageCount>
<abstractWordCount>169</abstractWordCount>
</qualityIndicators>
<title>Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3</title>
<genre><json:string>brief communication</json:string>
</genre>
<host><volume>26</volume>
<pages><total>5</total>
<last>735</last>
<first>731</first>
</pages>
<issn><json:string>0885-3185</json:string>
</issn>
<issue>4</issue>
<subject><json:item><value>Brief Report</value>
</json:item>
</subject>
<genre></genre>
<language><json:string>unknown</json:string>
</language>
<eissn><json:string>1531-8257</json:string>
</eissn>
<title>Movement Disorders</title>
<doi><json:string>10.1002/(ISSN)1531-8257</json:string>
</doi>
</host>
<publicationDate>2011</publicationDate>
<copyrightDate>2011</copyrightDate>
<doi><json:string>10.1002/mds.23428</json:string>
</doi>
<id>6E46E47B73A2D590EF06FE38F46F2D686EDA1122</id>
<fulltext><json:item><original>true</original>
<mimetype>application/pdf</mimetype>
<extension>pdf</extension>
<uri>https://api.istex.fr/document/6E46E47B73A2D590EF06FE38F46F2D686EDA1122/fulltext/pdf</uri>
</json:item>
<json:item><original>false</original>
<mimetype>application/zip</mimetype>
<extension>zip</extension>
<uri>https://api.istex.fr/document/6E46E47B73A2D590EF06FE38F46F2D686EDA1122/fulltext/zip</uri>
</json:item>
<istex:fulltextTEI uri="https://api.istex.fr/document/6E46E47B73A2D590EF06FE38F46F2D686EDA1122/fulltext/tei"><teiHeader><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3</title>
</titleStmt>
<publicationStmt><authority>ISTEX</authority>
<publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<pubPlace>Hoboken</pubPlace>
<availability><p>WILEY</p>
</availability>
<date>2011</date>
</publicationStmt>
<notesStmt><note type="content">*Potential conflict of interest: Nothing to report.</note>
<note>Brazilian funding agencies FIPE‐HCPA</note>
<note>CNPq</note>
<note>FINEP</note>
<note>INAGEMP</note>
<note>FAPERGS</note>
<note>Spanish institute CIBERNED</note>
</notesStmt>
<sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3</title>
<author><persName><forename type="first">Jonas Alex Morales</forename>
<surname>Saute</surname>
</persName>
<roleName type="degree">MD</roleName>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Neurology Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
</author>
<author><persName><forename type="first">Andrew Chaves Feitosa</forename>
<surname>da Silva</surname>
</persName>
<roleName type="degree">MD</roleName>
<affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
</author>
<author><persName><forename type="first">Alexandre Pastoris</forename>
<surname>Muller</surname>
</persName>
<roleName type="degree">MSc</roleName>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
</author>
<author><persName><forename type="first">Gisele</forename>
<surname>Hansel</surname>
</persName>
<roleName type="degree">MSc</roleName>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
</author>
<author><persName><forename type="first">Alexandre Silva</forename>
<surname>de Mello</surname>
</persName>
<roleName type="degree">MSc</roleName>
<affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
</author>
<author><persName><forename type="first">Fábio</forename>
<surname>Maeda</surname>
</persName>
<roleName type="degree">PhD</roleName>
<affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</affiliation>
</author>
<author><persName><forename type="first">Leonardo</forename>
<surname>Vedolin</surname>
</persName>
<roleName type="degree">MD, PhD</roleName>
<affiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</affiliation>
</author>
<author><persName><forename type="first">Maria Luiza</forename>
<surname>Saraiva‐Pereira</surname>
</persName>
<roleName type="degree">PhD</roleName>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
</author>
<author><persName><forename type="first">Diogo Onofre</forename>
<surname>Souza</surname>
</persName>
<roleName type="degree">MD, PhD</roleName>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
</author>
<author><persName><forename type="first">Javier</forename>
<surname>Arpa</surname>
</persName>
<roleName type="degree">MD</roleName>
<affiliation>Neurology Service, Hospital Universitario “La Paz” (HULP), Madrid, Spain</affiliation>
</author>
<author><persName><forename type="first">Ignacio</forename>
<surname>Torres‐Aleman</surname>
</persName>
<roleName type="degree">PhD</roleName>
<affiliation>Laboratory of Cellular and Molecular Neuroendocrinology, Instituto Cajal, Consejo Superior de Investigaciones Cientificas (CSIC), Madrid, Spain</affiliation>
<affiliation>CIBERNED, Madrid, Spain</affiliation>
</author>
<author><persName><forename type="first">Luis Valmor Cruz</forename>
<surname>Portela</surname>
</persName>
<roleName type="degree">PhD</roleName>
<note type="correspondence"><p>Correspondence: Department of Biochemistry, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600 Anexo, CEP 90.035‐003, Porto Alegre, Rio Grande do Sul, Brazil</p>
</note>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
</author>
<author><persName><forename type="first">Laura Bannach</forename>
<surname>Jardim</surname>
</persName>
<roleName type="degree">MD, PhD</roleName>
<affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
<affiliation>Department of Internal Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
</author>
</analytic>
<monogr><title level="j">Movement Disorders</title>
<title level="j" type="abbrev">Mov. Disord.</title>
<idno type="pISSN">0885-3185</idno>
<idno type="eISSN">1531-8257</idno>
<idno type="DOI">10.1002/(ISSN)1531-8257</idno>
<imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<pubPlace>Hoboken</pubPlace>
<date type="published" when="2011-03"></date>
<biblScope unit="volume">26</biblScope>
<biblScope unit="issue">4</biblScope>
<biblScope unit="page" from="731">731</biblScope>
<biblScope unit="page" to="735">735</biblScope>
</imprint>
</monogr>
<idno type="istex">6E46E47B73A2D590EF06FE38F46F2D686EDA1122</idno>
<idno type="DOI">10.1002/mds.23428</idno>
<idno type="ArticleID">MDS23428</idno>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><creation><date>2011</date>
</creation>
<langUsage><language ident="en">en</language>
</langUsage>
<abstract xml:lang="en"><p>Spinocerebellar ataxias (SCAs) constitute a group of autosomal dominant neurodegenerative disorders with no current treatment. The insulin/insulin‐like growth factor 1 (IGF‐1) system (IIS) has been shown to play a role in the neurological dysfunction of SCAs and other polyglutamine disorders. We aimed to study the biomarker profile of serum IIS components in SCA3. We performed a case–control study with 46 SCA3 patients and 42 healthy individuals evaluating the peripheral IIS profile (insulin, IGF‐1, IGFBP1 and 3) and the correlation with clinical, molecular, and neuroimaging findings. SCA3 patients presented lower insulin and IGFBP3 levels and higher insulin sensitivity (HOMA2), free IGF‐I, and IGFBP1 levels when compared with controls. IGFBP‐1 levels were directly associated with CAG expanded repeat length; IGF‐1 was associated with the volumetries of specific brainstem regions on magnetic resonance imaging (MRI). Insulin levels and sensitivity were related to age at onset of symptoms. Our findings indicate an involvement of IIS components in SCA3 neurobiology and IGFBP‐1 as a potential biomarker of the disease. © 2010 Movement Disorder Society</p>
</abstract>
<textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head>
<item><term>spinocerebellar ataxia</term>
</item>
<item><term>polyglutamine disorders</term>
</item>
<item><term>IGF‐1</term>
</item>
<item><term>insulin</term>
</item>
<item><term>IGFBP</term>
</item>
<item><term>Machado‐Joseph disease</term>
</item>
</list>
</keywords>
</textClass>
<textClass><keywords scheme="Journal Subject"><list><head>article category</head>
<item><term>Brief Report</term>
</item>
</list>
</keywords>
</textClass>
</profileDesc>
<revisionDesc><change when="2010-03-03">Received</change>
<change when="2010-08-11">Registration</change>
<change when="2011-03">Published</change>
</revisionDesc>
</teiHeader>
</istex:fulltextTEI>
<json:item><original>false</original>
<mimetype>text/plain</mimetype>
<extension>txt</extension>
<uri>https://api.istex.fr/document/6E46E47B73A2D590EF06FE38F46F2D686EDA1122/fulltext/txt</uri>
</json:item>
</fulltext>
<metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration>
<istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Wiley Subscription Services, Inc., A Wiley Company</publisherName>
<publisherLoc>Hoboken</publisherLoc>
</publisherInfo>
<doi registered="yes">10.1002/(ISSN)1531-8257</doi>
<issn type="print">0885-3185</issn>
<issn type="electronic">1531-8257</issn>
<idGroup><id type="product" value="MDS"></id>
</idGroup>
<titleGroup><title type="main" xml:lang="en" sort="MOVEMENT DISORDERS">Movement Disorders</title>
<title type="short">Mov. Disord.</title>
</titleGroup>
</publicationMeta>
<publicationMeta level="part" position="40"><doi origin="wiley" registered="yes">10.1002/mds.v26.4</doi>
<numberingGroup><numbering type="journalVolume" number="26">26</numbering>
<numbering type="journalIssue">4</numbering>
</numberingGroup>
<coverDate startDate="2011-03">March 2011</coverDate>
</publicationMeta>
<publicationMeta level="unit" type="shortCommunication" position="280" status="forIssue"><doi origin="wiley" registered="yes">10.1002/mds.23428</doi>
<idGroup><id type="unit" value="MDS23428"></id>
</idGroup>
<countGroup><count type="pageTotal" number="5"></count>
</countGroup>
<titleGroup><title type="articleCategory">Brief Report</title>
<title type="tocHeading1">Brief Reports</title>
</titleGroup>
<copyright ownership="thirdParty">Copyright © 2010 Movement Disorder Society</copyright>
<eventGroup><event type="manuscriptReceived" date="2010-03-03"></event>
<event type="manuscriptRevised" date="2010-04-13"></event>
<event type="manuscriptAccepted" date="2010-08-11"></event>
<event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.5.2 mode:FullText" date="2011-07-19"></event>
<event type="publishedOnlineEarlyUnpaginated" date="2010-11-10"></event>
<event type="publishedOnlineFinalForm" date="2011-04-19"></event>
<event type="firstOnline" date="2010-11-10"></event>
<event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.8.8" date="2014-02-02"></event>
<event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-10-31"></event>
</eventGroup>
<numberingGroup><numbering type="pageFirst">731</numbering>
<numbering type="pageLast">735</numbering>
</numberingGroup>
<correspondenceTo>Department of Biochemistry, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600 Anexo, CEP 90.035‐003, Porto Alegre, Rio Grande do Sul, Brazil</correspondenceTo>
<linkGroup><link type="toTypesetVersion" href="file:MDS.MDS23428.pdf"></link>
</linkGroup>
</publicationMeta>
<contentMeta><countGroup><count type="figureTotal" number="2"></count>
<count type="tableTotal" number="2"></count>
<count type="referenceTotal" number="22"></count>
<count type="wordTotal" number="3539"></count>
</countGroup>
<titleGroup><title type="main" xml:lang="en">Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3<link href="#fn5"></link>
</title>
<title type="short" xml:lang="en">Insulin‐Like System in SCA3</title>
</titleGroup>
<creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1 #af2 #af3"><personName><givenNames>Jonas Alex Morales</givenNames>
<familyName>Saute</familyName>
<degrees>MD</degrees>
</personName>
</creator>
<creator xml:id="au2" creatorRole="author" affiliationRef="#af4"><personName><givenNames>Andrew Chaves Feitosa</givenNames>
<familyName>da Silva</familyName>
<degrees>MD</degrees>
</personName>
</creator>
<creator xml:id="au3" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Alexandre Pastoris</givenNames>
<familyName>Muller</familyName>
<degrees>MSc</degrees>
</personName>
</creator>
<creator xml:id="au4" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Gisele</givenNames>
<familyName>Hansel</familyName>
<degrees>MSc</degrees>
</personName>
</creator>
<creator xml:id="au5" creatorRole="author" affiliationRef="#af4"><personName><givenNames>Alexandre Silva</givenNames>
<familyName>de Mello</familyName>
<degrees>MSc</degrees>
</personName>
</creator>
<creator xml:id="au6" creatorRole="author" affiliationRef="#af2 #af5"><personName><givenNames>Fábio</givenNames>
<familyName>Maeda</familyName>
<degrees>PhD</degrees>
</personName>
</creator>
<creator xml:id="au7" creatorRole="author" affiliationRef="#af5"><personName><givenNames>Leonardo</givenNames>
<familyName>Vedolin</familyName>
<degrees>MD, PhD</degrees>
</personName>
</creator>
<creator xml:id="au8" creatorRole="author" affiliationRef="#af1 #af4"><personName><givenNames>Maria Luiza</givenNames>
<familyName>Saraiva‐Pereira</familyName>
<degrees>PhD</degrees>
</personName>
</creator>
<creator xml:id="au9" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Diogo Onofre</givenNames>
<familyName>Souza</familyName>
<degrees>MD, PhD</degrees>
</personName>
</creator>
<creator xml:id="au10" creatorRole="author" affiliationRef="#af6"><personName><givenNames>Javier</givenNames>
<familyName>Arpa</familyName>
<degrees>MD</degrees>
</personName>
</creator>
<creator xml:id="au11" creatorRole="author" affiliationRef="#af7 #af8"><personName><givenNames>Ignacio</givenNames>
<familyName>Torres‐Aleman</familyName>
<degrees>PhD</degrees>
</personName>
</creator>
<creator xml:id="au12" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Luis Valmor Cruz</givenNames>
<familyName>Portela</familyName>
<degrees>PhD</degrees>
</personName>
<contactDetails><email>roskaportela@gmail.com</email>
</contactDetails>
</creator>
<creator xml:id="au13" creatorRole="author" affiliationRef="#af2 #af4 #af9"><personName><givenNames>Laura Bannach</givenNames>
<familyName>Jardim</familyName>
<degrees>MD, PhD</degrees>
</personName>
</creator>
</creators>
<affiliationGroup><affiliation xml:id="af1" countryCode="BR" type="organization"><unparsedAffiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af2" countryCode="BR" type="organization"><unparsedAffiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af3" countryCode="BR" type="organization"><unparsedAffiliation>Neurology Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af4" countryCode="BR" type="organization"><unparsedAffiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af5" countryCode="BR" type="organization"><unparsedAffiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af6" countryCode="ES" type="organization"><unparsedAffiliation>Neurology Service, Hospital Universitario “La Paz” (HULP), Madrid, Spain</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af7" countryCode="ES" type="organization"><unparsedAffiliation>Laboratory of Cellular and Molecular Neuroendocrinology, Instituto Cajal, Consejo Superior de Investigaciones Cientificas (CSIC), Madrid, Spain</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af8" countryCode="ES" type="organization"><unparsedAffiliation>CIBERNED, Madrid, Spain</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af9" countryCode="BR" type="organization"><unparsedAffiliation>Department of Internal Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</unparsedAffiliation>
</affiliation>
</affiliationGroup>
<keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">spinocerebellar ataxia</keyword>
<keyword xml:id="kwd2">polyglutamine disorders</keyword>
<keyword xml:id="kwd3">IGF‐1</keyword>
<keyword xml:id="kwd4">insulin</keyword>
<keyword xml:id="kwd5">IGFBP</keyword>
<keyword xml:id="kwd6">Machado‐Joseph disease</keyword>
</keywordGroup>
<fundingInfo><fundingAgency>Brazilian funding agencies FIPE‐HCPA</fundingAgency>
</fundingInfo>
<fundingInfo><fundingAgency>CNPq</fundingAgency>
</fundingInfo>
<fundingInfo><fundingAgency>FINEP</fundingAgency>
</fundingInfo>
<fundingInfo><fundingAgency>INAGEMP</fundingAgency>
</fundingInfo>
<fundingInfo><fundingAgency>FAPERGS</fundingAgency>
</fundingInfo>
<fundingInfo><fundingAgency>Spanish institute CIBERNED</fundingAgency>
</fundingInfo>
<supportingInformation><p> Additional Supporting Information may be found in the online version of this article. </p>
<supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:08853185:media:mds23428:MDS_23428_sm_SuppFig1"></mediaResource>
<caption>Supporting Information Figure 1. The inverse correlation between IGF‐1 serum levels and Basis Pons and Medulla Oblongata volumetries on MRI. Two patients with similar ages, disease duration and CAG expanded repeats were chosen as an example. (A) and (C) represent a fifty two year old man, with nine years of disease duration and 72 CAG repeats on MJD gene. (B) and (D) represent a fifty one year old woman, with thirteen years of disease duration and 71 CAG repeats on MJD gene. Bars indicate percent difference between the individual and the mean study population values of IGF‐1 serum levels and MRI brainstem volumes.</caption>
</supportingInfoItem>
<supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:08853185:media:mds23428:MDS_23428_sm_SuppFig2"></mediaResource>
<caption>Supporting Information Figure 2. Total IGF‐1 correlations with brainstem volumetries. IGF‐1 serum levels simple correlation with (A) medulla oblongata and (B) basis of pons MRI volumetries.* p<0.05.</caption>
</supportingInfoItem>
</supportingInformation>
<abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title>
<p>Spinocerebellar ataxias (SCAs) constitute a group of autosomal dominant neurodegenerative disorders with no current treatment. The insulin/insulin‐like growth factor 1 (IGF‐1) system (IIS) has been shown to play a role in the neurological dysfunction of SCAs and other polyglutamine disorders. We aimed to study the biomarker profile of serum IIS components in SCA3. We performed a case–control study with 46 SCA3 patients and 42 healthy individuals evaluating the peripheral IIS profile (insulin, IGF‐1, IGFBP1 and 3) and the correlation with clinical, molecular, and neuroimaging findings. SCA3 patients presented lower insulin and IGFBP3 levels and higher insulin sensitivity (HOMA2), free IGF‐I, and IGFBP1 levels when compared with controls. IGFBP‐1 levels were directly associated with CAG expanded repeat length; IGF‐1 was associated with the volumetries of specific brainstem regions on magnetic resonance imaging (MRI). Insulin levels and sensitivity were related to age at onset of symptoms. Our findings indicate an involvement of IIS components in SCA3 neurobiology and IGFBP‐1 as a potential biomarker of the disease. © 2010 Movement Disorder Society</p>
</abstract>
</abstractGroup>
</contentMeta>
<noteGroup><note xml:id="fn5"><p>Potential conflict of interest: Nothing to report.</p>
</note>
</noteGroup>
</header>
</component>
</istex:document>
</istex:metadataXml>
<mods version="3.6"><titleInfo lang="en"><title>Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3</title>
</titleInfo>
<titleInfo type="abbreviated" lang="en"><title>Insulin‐Like System in SCA3</title>
</titleInfo>
<titleInfo type="alternative" contentType="CDATA" lang="en"><title>Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3</title>
</titleInfo>
<name type="personal"><namePart type="given">Jonas Alex Morales</namePart>
<namePart type="family">Saute</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Neurology Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Andrew Chaves Feitosa</namePart>
<namePart type="family">da Silva</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Alexandre Pastoris</namePart>
<namePart type="family">Muller</namePart>
<namePart type="termsOfAddress">MSc</namePart>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Gisele</namePart>
<namePart type="family">Hansel</namePart>
<namePart type="termsOfAddress">MSc</namePart>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Alexandre Silva</namePart>
<namePart type="family">de Mello</namePart>
<namePart type="termsOfAddress">MSc</namePart>
<affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Fábio</namePart>
<namePart type="family">Maeda</namePart>
<namePart type="termsOfAddress">PhD</namePart>
<affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Leonardo</namePart>
<namePart type="family">Vedolin</namePart>
<namePart type="termsOfAddress">MD, PhD</namePart>
<affiliation>Neuroradiology Service, Hospital Moinhos de Vento, Porto Alegre, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Maria Luiza</namePart>
<namePart type="family">Saraiva‐Pereira</namePart>
<namePart type="termsOfAddress">PhD</namePart>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Diogo Onofre</namePart>
<namePart type="family">Souza</namePart>
<namePart type="termsOfAddress">MD, PhD</namePart>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Javier</namePart>
<namePart type="family">Arpa</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Neurology Service, Hospital Universitario “La Paz” (HULP), Madrid, Spain</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Ignacio</namePart>
<namePart type="family">Torres‐Aleman</namePart>
<namePart type="termsOfAddress">PhD</namePart>
<affiliation>Laboratory of Cellular and Molecular Neuroendocrinology, Instituto Cajal, Consejo Superior de Investigaciones Cientificas (CSIC), Madrid, Spain</affiliation>
<affiliation>CIBERNED, Madrid, Spain</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Luis Valmor Cruz</namePart>
<namePart type="family">Portela</namePart>
<namePart type="termsOfAddress">PhD</namePart>
<affiliation>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<description>Correspondence: Department of Biochemistry, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600 Anexo, CEP 90.035‐003, Porto Alegre, Rio Grande do Sul, Brazil</description>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Laura Bannach</namePart>
<namePart type="family">Jardim</namePart>
<namePart type="termsOfAddress">MD, PhD</namePart>
<affiliation>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<affiliation>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil</affiliation>
<affiliation>Department of Internal Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<typeOfResource>text</typeOfResource>
<genre type="brief communication">shortCommunication</genre>
<originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<place><placeTerm type="text">Hoboken</placeTerm>
</place>
<dateIssued encoding="w3cdtf">2011-03</dateIssued>
<dateCaptured encoding="w3cdtf">2010-03-03</dateCaptured>
<dateValid encoding="w3cdtf">2010-08-11</dateValid>
<copyrightDate encoding="w3cdtf">2011</copyrightDate>
</originInfo>
<language><languageTerm type="code" authority="rfc3066">en</languageTerm>
<languageTerm type="code" authority="iso639-2b">eng</languageTerm>
</language>
<physicalDescription><internetMediaType>text/html</internetMediaType>
<extent unit="figures">2</extent>
<extent unit="tables">2</extent>
<extent unit="references">22</extent>
<extent unit="words">3539</extent>
</physicalDescription>
<abstract lang="en">Spinocerebellar ataxias (SCAs) constitute a group of autosomal dominant neurodegenerative disorders with no current treatment. The insulin/insulin‐like growth factor 1 (IGF‐1) system (IIS) has been shown to play a role in the neurological dysfunction of SCAs and other polyglutamine disorders. We aimed to study the biomarker profile of serum IIS components in SCA3. We performed a case–control study with 46 SCA3 patients and 42 healthy individuals evaluating the peripheral IIS profile (insulin, IGF‐1, IGFBP1 and 3) and the correlation with clinical, molecular, and neuroimaging findings. SCA3 patients presented lower insulin and IGFBP3 levels and higher insulin sensitivity (HOMA2), free IGF‐I, and IGFBP1 levels when compared with controls. IGFBP‐1 levels were directly associated with CAG expanded repeat length; IGF‐1 was associated with the volumetries of specific brainstem regions on magnetic resonance imaging (MRI). Insulin levels and sensitivity were related to age at onset of symptoms. Our findings indicate an involvement of IIS components in SCA3 neurobiology and IGFBP‐1 as a potential biomarker of the disease. © 2010 Movement Disorder Society</abstract>
<note type="content">*Potential conflict of interest: Nothing to report.</note>
<note type="funding">Brazilian funding agencies FIPE‐HCPA</note>
<note type="funding">CNPq</note>
<note type="funding">FINEP</note>
<note type="funding">INAGEMP</note>
<note type="funding">FAPERGS</note>
<note type="funding">Spanish institute CIBERNED</note>
<subject lang="en"><genre>Keywords</genre>
<topic>spinocerebellar ataxia</topic>
<topic>polyglutamine disorders</topic>
<topic>IGF‐1</topic>
<topic>insulin</topic>
<topic>IGFBP</topic>
<topic>Machado‐Joseph disease</topic>
</subject>
<relatedItem type="host"><titleInfo><title>Movement Disorders</title>
</titleInfo>
<titleInfo type="abbreviated"><title>Mov. Disord.</title>
</titleInfo>
<note type="content"> Additional Supporting Information may be found in the online version of this article.Supporting Info Item: Supporting Information Figure 1. The inverse correlation between IGF‐1 serum levels and Basis Pons and Medulla Oblongata volumetries on MRI. Two patients with similar ages, disease duration and CAG expanded repeats were chosen as an example. (A) and (C) represent a fifty two year old man, with nine years of disease duration and 72 CAG repeats on MJD gene. (B) and (D) represent a fifty one year old woman, with thirteen years of disease duration and 71 CAG repeats on MJD gene. Bars indicate percent difference between the individual and the mean study population values of IGF‐1 serum levels and MRI brainstem volumes. - Supporting Information Figure 2. Total IGF‐1 correlations with brainstem volumetries. IGF‐1 serum levels simple correlation with (A) medulla oblongata and (B) basis of pons MRI volumetries.* p<0.05. - </note>
<subject><genre>article category</genre>
<topic>Brief Report</topic>
</subject>
<identifier type="ISSN">0885-3185</identifier>
<identifier type="eISSN">1531-8257</identifier>
<identifier type="DOI">10.1002/(ISSN)1531-8257</identifier>
<identifier type="PublisherID">MDS</identifier>
<part><date>2011</date>
<detail type="volume"><caption>vol.</caption>
<number>26</number>
</detail>
<detail type="issue"><caption>no.</caption>
<number>4</number>
</detail>
<extent unit="pages"><start>731</start>
<end>735</end>
<total>5</total>
</extent>
</part>
</relatedItem>
<identifier type="istex">6E46E47B73A2D590EF06FE38F46F2D686EDA1122</identifier>
<identifier type="DOI">10.1002/mds.23428</identifier>
<identifier type="ArticleID">MDS23428</identifier>
<accessCondition type="use and reproduction" contentType="copyright">Copyright © 2010 Movement Disorder Society</accessCondition>
<recordInfo><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin>
<recordContentSource>WILEY</recordContentSource>
</recordInfo>
</mods>
</metadata>
<serie></serie>
</istex>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Santé/explor/JankovicV1/Data/Istex/Corpus
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000C40 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Istex/Corpus/biblio.hfd -nk 000C40 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Santé |area= JankovicV1 |flux= Istex |étape= Corpus |type= RBID |clé= ISTEX:6E46E47B73A2D590EF06FE38F46F2D686EDA1122 |texte= Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3 }}
![]() | This area was generated with Dilib version V0.6.19. | ![]() |