Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3
Identifieur interne : 001390 ( Main/Exploration ); précédent : 001389; suivant : 001391Serum insulin‐like system alterations in patients with spinocerebellar ataxia type 3
Auteurs : Jonas Alex Morales Saute [Brésil] ; Andrew Chaves Feitosa Da Silva [Brésil] ; Alexandre Pastoris Muller [Brésil] ; Gisele Hansel [Brésil] ; Alexandre Silva De Mello [Brésil] ; Fábio Maeda [Brésil] ; Leonardo Vedolin [Brésil] ; Maria Luiza Saraiva-Pereira [Brésil] ; Diogo Onofre Souza [Brésil] ; Javier Arpa [Espagne] ; Ignacio Torres-Aleman [Espagne] ; Luis Valmor Cruz Portela [Brésil] ; Laura Bannach Jardim [Brésil]Source :
- Movement Disorders [ 0885-3185 ] ; 2011-03.
English descriptors
- KwdEn :
- Adult, Case-Control Studies, Female, Humans, IGFBP, IGF‐1, Insulin (blood), Insulin-Like Growth Factor Binding Protein 1 (blood), Insulin-Like Growth Factor Binding Protein 3, Insulin-Like Growth Factor Binding Proteins (blood), Insulin-Like Growth Factor I (metabolism), Machado-Joseph Disease (blood), Machado-Joseph Disease (genetics), Machado‐Joseph disease, Male, Middle Aged, Nerve Tissue Proteins (genetics), Nuclear Proteins (genetics), Repressor Proteins (genetics), insulin, polyglutamine disorders, spinocerebellar ataxia.
- MESH :
- chemical , blood : Insulin, Insulin-Like Growth Factor Binding Protein 1, Insulin-Like Growth Factor Binding Proteins.
- chemical , genetics : Nerve Tissue Proteins, Nuclear Proteins, Repressor Proteins.
- chemical , metabolism : Insulin-Like Growth Factor I.
- blood : Machado-Joseph Disease.
- genetics : Machado-Joseph Disease.
- Adult, Case-Control Studies, Female, Humans, Insulin-Like Growth Factor Binding Protein 3, Male, Middle Aged.
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
Affiliations:
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Sul</region></placeName></affiliation></author><author><name sortKey="Arpa, Javier" sort="Arpa, Javier" uniqKey="Arpa J" first="Javier" last="Arpa">Javier Arpa</name><affiliation wicri:level="3"><country xml:lang="fr">Espagne</country><wicri:regionArea>Neurology Service, Hospital Universitario “La Paz” (HULP), Madrid</wicri:regionArea><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Torres Leman, Ignacio" sort="Torres Leman, Ignacio" uniqKey="Torres Leman I" first="Ignacio" last="Torres-Aleman">Ignacio Torres-Aleman</name><affiliation wicri:level="3"><country xml:lang="fr">Espagne</country><wicri:regionArea>Laboratory of Cellular and Molecular Neuroendocrinology, Instituto Cajal, Consejo Superior de Investigaciones Cientificas (CSIC), Madrid</wicri:regionArea><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation><affiliation wicri:level="3"><country xml:lang="fr">Espagne</country><wicri:regionArea>CIBERNED, Madrid</wicri:regionArea><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Portela, Luis Valmor Cruz" sort="Portela, Luis Valmor Cruz" uniqKey="Portela L" first="Luis Valmor Cruz" last="Portela">Luis Valmor Cruz Portela</name><affiliation wicri:level="2"><country xml:lang="fr">Brésil</country><wicri:regionArea>Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS</wicri:regionArea><placeName><region type="state">Rio Grande do Sul</region></placeName></affiliation></author><author><name sortKey="Jardim, Laura Bannach" sort="Jardim, Laura Bannach" uniqKey="Jardim L" first="Laura Bannach" last="Jardim">Laura Bannach Jardim</name><affiliation wicri:level="2"><country xml:lang="fr">Brésil</country><wicri:regionArea>Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS</wicri:regionArea><placeName><region type="state">Rio Grande do Sul</region></placeName></affiliation><affiliation wicri:level="2"><country xml:lang="fr">Brésil</country><wicri:regionArea>Medical Genetics Service, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS</wicri:regionArea><placeName><region type="state">Rio Grande do Sul</region></placeName></affiliation><affiliation wicri:level="2"><country xml:lang="fr">Brésil</country><wicri:regionArea>Department of Internal Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS</wicri:regionArea><placeName><region type="state">Rio Grande do Sul</region></placeName></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="vol">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>Adult</term><term>Case-Control Studies</term><term>Female</term><term>Humans</term><term>IGFBP</term><term>IGF‐1</term><term>Insulin (blood)</term><term>Insulin-Like Growth Factor Binding Protein 1 (blood)</term><term>Insulin-Like Growth Factor Binding Protein 3</term><term>Insulin-Like Growth Factor Binding Proteins (blood)</term><term>Insulin-Like Growth Factor I (metabolism)</term><term>Machado-Joseph Disease (blood)</term><term>Machado-Joseph Disease (genetics)</term><term>Machado‐Joseph disease</term><term>Male</term><term>Middle Aged</term><term>Nerve Tissue Proteins (genetics)</term><term>Nuclear Proteins (genetics)</term><term>Repressor Proteins (genetics)</term><term>insulin</term><term>polyglutamine disorders</term><term>spinocerebellar ataxia</term></keywords><keywords scheme="MESH" type="chemical" qualifier="blood" xml:lang="en"><term>Insulin</term><term>Insulin-Like Growth Factor Binding Protein 1</term><term>Insulin-Like Growth Factor Binding Proteins</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Nerve Tissue Proteins</term><term>Nuclear Proteins</term><term>Repressor Proteins</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Insulin-Like Growth Factor I</term></keywords><keywords scheme="MESH" qualifier="blood" xml:lang="en"><term>Machado-Joseph Disease</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Machado-Joseph Disease</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Case-Control Studies</term><term>Female</term><term>Humans</term><term>Insulin-Like Growth Factor Binding Protein 3</term><term>Male</term><term>Middle Aged</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><affiliations><list><country><li>Brésil</li><li>Espagne</li></country><region><li>Communauté de Madrid</li><li>Rio Grande do Sul</li></region><settlement><li>Madrid</li><li>Porto Alegre</li></settlement></list><tree><country name="Brésil"><region name="Rio Grande do Sul"><name sortKey="Saute, Jonas Alex Morales" sort="Saute, Jonas Alex Morales" uniqKey="Saute J" first="Jonas Alex Morales" last="Saute">Jonas Alex Morales Saute</name></region><name sortKey="Da Silva, Andrew Chaves Feitosa" sort="Da Silva, Andrew Chaves Feitosa" uniqKey="Da Silva A" first="Andrew Chaves Feitosa" last="Da Silva">Andrew Chaves Feitosa Da Silva</name><name sortKey="De Mello, Alexandre Silva" sort="De Mello, Alexandre Silva" uniqKey="De Mello A" first="Alexandre Silva" last="De Mello">Alexandre Silva De Mello</name><name sortKey="Hansel, Gisele" sort="Hansel, Gisele" uniqKey="Hansel G" first="Gisele" last="Hansel">Gisele Hansel</name><name sortKey="Jardim, Laura Bannach" sort="Jardim, Laura Bannach" uniqKey="Jardim L" first="Laura Bannach" last="Jardim">Laura Bannach Jardim</name><name sortKey="Jardim, Laura Bannach" sort="Jardim, Laura Bannach" uniqKey="Jardim L" first="Laura Bannach" last="Jardim">Laura Bannach Jardim</name><name sortKey="Jardim, Laura Bannach" sort="Jardim, Laura Bannach" uniqKey="Jardim L" first="Laura Bannach" last="Jardim">Laura Bannach Jardim</name><name sortKey="Maeda, Fabio" sort="Maeda, Fabio" uniqKey="Maeda F" first="Fábio" last="Maeda">Fábio Maeda</name><name sortKey="Maeda, Fabio" sort="Maeda, Fabio" uniqKey="Maeda F" first="Fábio" last="Maeda">Fábio Maeda</name><name sortKey="Muller, Alexandre Pastoris" sort="Muller, Alexandre Pastoris" uniqKey="Muller A" first="Alexandre Pastoris" last="Muller">Alexandre Pastoris Muller</name><name sortKey="Portela, Luis Valmor Cruz" sort="Portela, Luis Valmor Cruz" uniqKey="Portela L" first="Luis Valmor Cruz" last="Portela">Luis Valmor Cruz Portela</name><name sortKey="Saraiva Ereira, Maria Luiza" sort="Saraiva Ereira, Maria Luiza" uniqKey="Saraiva Ereira M" first="Maria Luiza" last="Saraiva-Pereira">Maria Luiza Saraiva-Pereira</name><name sortKey="Saraiva Ereira, Maria Luiza" sort="Saraiva Ereira, Maria Luiza" uniqKey="Saraiva Ereira M" first="Maria Luiza" last="Saraiva-Pereira">Maria Luiza Saraiva-Pereira</name><name sortKey="Saute, Jonas Alex Morales" sort="Saute, Jonas Alex Morales" uniqKey="Saute J" first="Jonas Alex Morales" last="Saute">Jonas Alex Morales Saute</name><name sortKey="Saute, Jonas Alex Morales" sort="Saute, Jonas Alex Morales" uniqKey="Saute J" first="Jonas Alex Morales" last="Saute">Jonas Alex Morales Saute</name><name sortKey="Souza, Diogo Onofre" sort="Souza, Diogo Onofre" uniqKey="Souza D" first="Diogo Onofre" last="Souza">Diogo Onofre Souza</name><name sortKey="Vedolin, Leonardo" sort="Vedolin, Leonardo" uniqKey="Vedolin L" first="Leonardo" last="Vedolin">Leonardo Vedolin</name></country><country name="Espagne"><region name="Communauté de Madrid"><name sortKey="Arpa, Javier" sort="Arpa, Javier" uniqKey="Arpa J" first="Javier" last="Arpa">Javier Arpa</name></region><name sortKey="Torres Leman, Ignacio" sort="Torres Leman, Ignacio" uniqKey="Torres Leman I" first="Ignacio" last="Torres-Aleman">Ignacio Torres-Aleman</name><name sortKey="Torres Leman, Ignacio" sort="Torres Leman, Ignacio" uniqKey="Torres Leman I" first="Ignacio" last="Torres-Aleman">Ignacio Torres-Aleman</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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