DPPX potassium channel antibody
Identifieur interne : 000B08 ( Main/Exploration ); précédent : 000B07; suivant : 000B09DPPX potassium channel antibody
Auteurs : William Oliver Tobin ; Vanda A. Lennon ; Lars Komorowski ; Christian Probst ; Stacey Lynn Clardy ; Allen J. Aksamit ; Juan Pablo Appendino ; Claudia F. Lucchinetti ; Joseph Y. Matsumoto ; Sean J. Pittock ; Paola Sandroni ; Maja Tippmann-Peikert ; Elaine C. Wirrell ; Andrew MckeonSource :
- Neurology [ 0028-3878 ] ; 2014.
Abstract
To describe the detection frequency and clinical associations of immunoglobulin G (IgG) targeting dipeptidyl-peptidase-like protein-6 (DPPX), a regulatory subunit of neuronal Kv4.2 potassium channels.
Specimens from 20 patients evaluated on a service basis by tissue-based immunofluorescence yielded a synaptic immunostaining pattern consistent with DPPX-IgG (serum, 20; CSF, all 7 available). Transfected HEK293 cell-based assay confirmed DPPX specificity in all specimens. Sixty-nine patients with stiff-person syndrome and related disorders were also evaluated by DPPX-IgG cell-based assay.
Of 20 seropositive patients, 12 were men; median symptom onset age was 53 years (range, 13–75). Symptom onset was insidious in 15 and subacute in 5. Twelve patients reported prodromal weight loss. Neurologic disorders were multifocal. All had one or more brain or brainstem manifestations: amnesia (16), delirium (8), psychosis (4), depression (4), seizures (2), and brainstem disorders (15; eye movement disturbances [8], ataxia [7], dysphagia [6], dysarthria [4], respiratory failure [3]). Nine patients reported sleep disturbance. Manifestations of central hyperexcitability included myoclonus (8), exaggerated startle (6), diffuse rigidity (6), and hyperreflexia (6). Dysautonomia involved the gastrointestinal tract (9; diarrhea [6], gastroparesis, and constipation [3]), bladder (7), cardiac conduction system (3), and thermoregulation (1). Two patients had B-cell neoplasms: gastrointestinal lymphoma (1), and chronic lymphocytic leukemia (1). Substantial neurologic improvements followed immunotherapy in 7 of 11 patients with available treatment data. DPPX-IgG was not detected in any of the stiff-person syndrome patients.
DPPX-IgG is a biomarker for an immunotherapy-responsive multifocal neurologic disorder of the central and autonomic nervous systems.
Url:
DOI: 10.1212/WNL.0000000000000991
PubMed: 25320100
PubMed Central: 4240433
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream Pmc, to step Corpus: 000619
- to stream Pmc, to step Curation: 000619
- to stream Pmc, to step Checkpoint: 000514
- to stream Ncbi, to step Merge: 001939
- to stream Ncbi, to step Curation: 001939
- to stream Ncbi, to step Checkpoint: 001939
- to stream Main, to step Merge: 000B12
- to stream Main, to step Curation: 000B08
Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">DPPX potassium channel antibody</title>
<author><name sortKey="Tobin, William Oliver" sort="Tobin, William Oliver" uniqKey="Tobin W" first="William Oliver" last="Tobin">William Oliver Tobin</name>
</author>
<author><name sortKey="Lennon, Vanda A" sort="Lennon, Vanda A" uniqKey="Lennon V" first="Vanda A." last="Lennon">Vanda A. Lennon</name>
</author>
<author><name sortKey="Komorowski, Lars" sort="Komorowski, Lars" uniqKey="Komorowski L" first="Lars" last="Komorowski">Lars Komorowski</name>
</author>
<author><name sortKey="Probst, Christian" sort="Probst, Christian" uniqKey="Probst C" first="Christian" last="Probst">Christian Probst</name>
</author>
<author><name sortKey="Clardy, Stacey Lynn" sort="Clardy, Stacey Lynn" uniqKey="Clardy S" first="Stacey Lynn" last="Clardy">Stacey Lynn Clardy</name>
</author>
<author><name sortKey="Aksamit, Allen J" sort="Aksamit, Allen J" uniqKey="Aksamit A" first="Allen J." last="Aksamit">Allen J. Aksamit</name>
</author>
<author><name sortKey="Appendino, Juan Pablo" sort="Appendino, Juan Pablo" uniqKey="Appendino J" first="Juan Pablo" last="Appendino">Juan Pablo Appendino</name>
</author>
<author><name sortKey="Lucchinetti, Claudia F" sort="Lucchinetti, Claudia F" uniqKey="Lucchinetti C" first="Claudia F." last="Lucchinetti">Claudia F. Lucchinetti</name>
</author>
<author><name sortKey="Matsumoto, Joseph Y" sort="Matsumoto, Joseph Y" uniqKey="Matsumoto J" first="Joseph Y." last="Matsumoto">Joseph Y. Matsumoto</name>
</author>
<author><name sortKey="Pittock, Sean J" sort="Pittock, Sean J" uniqKey="Pittock S" first="Sean J." last="Pittock">Sean J. Pittock</name>
</author>
<author><name sortKey="Sandroni, Paola" sort="Sandroni, Paola" uniqKey="Sandroni P" first="Paola" last="Sandroni">Paola Sandroni</name>
</author>
<author><name sortKey="Tippmann Peikert, Maja" sort="Tippmann Peikert, Maja" uniqKey="Tippmann Peikert M" first="Maja" last="Tippmann-Peikert">Maja Tippmann-Peikert</name>
</author>
<author><name sortKey="Wirrell, Elaine C" sort="Wirrell, Elaine C" uniqKey="Wirrell E" first="Elaine C." last="Wirrell">Elaine C. Wirrell</name>
</author>
<author><name sortKey="Mckeon, Andrew" sort="Mckeon, Andrew" uniqKey="Mckeon A" first="Andrew" last="Mckeon">Andrew Mckeon</name>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PMC</idno>
<idno type="pmid">25320100</idno>
<idno type="pmc">4240433</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4240433</idno>
<idno type="RBID">PMC:4240433</idno>
<idno type="doi">10.1212/WNL.0000000000000991</idno>
<date when="2014">2014</date>
<idno type="wicri:Area/Pmc/Corpus">000619</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000619</idno>
<idno type="wicri:Area/Pmc/Curation">000619</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">000619</idno>
<idno type="wicri:Area/Pmc/Checkpoint">000514</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">000514</idno>
<idno type="wicri:Area/Ncbi/Merge">001939</idno>
<idno type="wicri:Area/Ncbi/Curation">001939</idno>
<idno type="wicri:Area/Ncbi/Checkpoint">001939</idno>
<idno type="wicri:doubleKey">0028-3878:2014:Tobin W:dppx:potassium:channel</idno>
<idno type="wicri:Area/Main/Merge">000B12</idno>
<idno type="wicri:Area/Main/Curation">000B08</idno>
<idno type="wicri:Area/Main/Exploration">000B08</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">DPPX potassium channel antibody</title>
<author><name sortKey="Tobin, William Oliver" sort="Tobin, William Oliver" uniqKey="Tobin W" first="William Oliver" last="Tobin">William Oliver Tobin</name>
</author>
<author><name sortKey="Lennon, Vanda A" sort="Lennon, Vanda A" uniqKey="Lennon V" first="Vanda A." last="Lennon">Vanda A. Lennon</name>
</author>
<author><name sortKey="Komorowski, Lars" sort="Komorowski, Lars" uniqKey="Komorowski L" first="Lars" last="Komorowski">Lars Komorowski</name>
</author>
<author><name sortKey="Probst, Christian" sort="Probst, Christian" uniqKey="Probst C" first="Christian" last="Probst">Christian Probst</name>
</author>
<author><name sortKey="Clardy, Stacey Lynn" sort="Clardy, Stacey Lynn" uniqKey="Clardy S" first="Stacey Lynn" last="Clardy">Stacey Lynn Clardy</name>
</author>
<author><name sortKey="Aksamit, Allen J" sort="Aksamit, Allen J" uniqKey="Aksamit A" first="Allen J." last="Aksamit">Allen J. Aksamit</name>
</author>
<author><name sortKey="Appendino, Juan Pablo" sort="Appendino, Juan Pablo" uniqKey="Appendino J" first="Juan Pablo" last="Appendino">Juan Pablo Appendino</name>
</author>
<author><name sortKey="Lucchinetti, Claudia F" sort="Lucchinetti, Claudia F" uniqKey="Lucchinetti C" first="Claudia F." last="Lucchinetti">Claudia F. Lucchinetti</name>
</author>
<author><name sortKey="Matsumoto, Joseph Y" sort="Matsumoto, Joseph Y" uniqKey="Matsumoto J" first="Joseph Y." last="Matsumoto">Joseph Y. Matsumoto</name>
</author>
<author><name sortKey="Pittock, Sean J" sort="Pittock, Sean J" uniqKey="Pittock S" first="Sean J." last="Pittock">Sean J. Pittock</name>
</author>
<author><name sortKey="Sandroni, Paola" sort="Sandroni, Paola" uniqKey="Sandroni P" first="Paola" last="Sandroni">Paola Sandroni</name>
</author>
<author><name sortKey="Tippmann Peikert, Maja" sort="Tippmann Peikert, Maja" uniqKey="Tippmann Peikert M" first="Maja" last="Tippmann-Peikert">Maja Tippmann-Peikert</name>
</author>
<author><name sortKey="Wirrell, Elaine C" sort="Wirrell, Elaine C" uniqKey="Wirrell E" first="Elaine C." last="Wirrell">Elaine C. Wirrell</name>
</author>
<author><name sortKey="Mckeon, Andrew" sort="Mckeon, Andrew" uniqKey="Mckeon A" first="Andrew" last="Mckeon">Andrew Mckeon</name>
</author>
</analytic>
<series><title level="j">Neurology</title>
<idno type="ISSN">0028-3878</idno>
<idno type="eISSN">1526-632X</idno>
<imprint><date when="2014">2014</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><sec><title>Objective:</title>
<p>To describe the detection frequency and clinical associations of immunoglobulin G (IgG) targeting dipeptidyl-peptidase-like protein-6 (DPPX), a regulatory subunit of neuronal Kv4.2 potassium channels.</p>
</sec>
<sec><title>Methods:</title>
<p>Specimens from 20 patients evaluated on a service basis by tissue-based immunofluorescence yielded a synaptic immunostaining pattern consistent with DPPX-IgG (serum, 20; CSF, all 7 available). Transfected HEK293 cell-based assay confirmed DPPX specificity in all specimens. Sixty-nine patients with stiff-person syndrome and related disorders were also evaluated by DPPX-IgG cell-based assay.</p>
</sec>
<sec><title>Results:</title>
<p>Of 20 seropositive patients, 12 were men; median symptom onset age was 53 years (range, 13–75). Symptom onset was insidious in 15 and subacute in 5. Twelve patients reported prodromal weight loss. Neurologic disorders were multifocal. All had one or more brain or brainstem manifestations: amnesia (16), delirium (8), psychosis (4), depression (4), seizures (2), and brainstem disorders (15; eye movement disturbances [8], ataxia [7], dysphagia [6], dysarthria [4], respiratory failure [3]). Nine patients reported sleep disturbance. Manifestations of central hyperexcitability included myoclonus (8), exaggerated startle (6), diffuse rigidity (6), and hyperreflexia (6). Dysautonomia involved the gastrointestinal tract (9; diarrhea [6], gastroparesis, and constipation [3]), bladder (7), cardiac conduction system (3), and thermoregulation (1). Two patients had B-cell neoplasms: gastrointestinal lymphoma (1), and chronic lymphocytic leukemia (1). Substantial neurologic improvements followed immunotherapy in 7 of 11 patients with available treatment data. DPPX-IgG was not detected in any of the stiff-person syndrome patients.</p>
</sec>
<sec><title>Conclusions:</title>
<p>DPPX-IgG is a biomarker for an immunotherapy-responsive multifocal neurologic disorder of the central and autonomic nervous systems.</p>
</sec>
</div>
</front>
</TEI>
<affiliations><list></list>
<tree><noCountry><name sortKey="Aksamit, Allen J" sort="Aksamit, Allen J" uniqKey="Aksamit A" first="Allen J." last="Aksamit">Allen J. Aksamit</name>
<name sortKey="Appendino, Juan Pablo" sort="Appendino, Juan Pablo" uniqKey="Appendino J" first="Juan Pablo" last="Appendino">Juan Pablo Appendino</name>
<name sortKey="Clardy, Stacey Lynn" sort="Clardy, Stacey Lynn" uniqKey="Clardy S" first="Stacey Lynn" last="Clardy">Stacey Lynn Clardy</name>
<name sortKey="Komorowski, Lars" sort="Komorowski, Lars" uniqKey="Komorowski L" first="Lars" last="Komorowski">Lars Komorowski</name>
<name sortKey="Lennon, Vanda A" sort="Lennon, Vanda A" uniqKey="Lennon V" first="Vanda A." last="Lennon">Vanda A. Lennon</name>
<name sortKey="Lucchinetti, Claudia F" sort="Lucchinetti, Claudia F" uniqKey="Lucchinetti C" first="Claudia F." last="Lucchinetti">Claudia F. Lucchinetti</name>
<name sortKey="Matsumoto, Joseph Y" sort="Matsumoto, Joseph Y" uniqKey="Matsumoto J" first="Joseph Y." last="Matsumoto">Joseph Y. Matsumoto</name>
<name sortKey="Mckeon, Andrew" sort="Mckeon, Andrew" uniqKey="Mckeon A" first="Andrew" last="Mckeon">Andrew Mckeon</name>
<name sortKey="Pittock, Sean J" sort="Pittock, Sean J" uniqKey="Pittock S" first="Sean J." last="Pittock">Sean J. Pittock</name>
<name sortKey="Probst, Christian" sort="Probst, Christian" uniqKey="Probst C" first="Christian" last="Probst">Christian Probst</name>
<name sortKey="Sandroni, Paola" sort="Sandroni, Paola" uniqKey="Sandroni P" first="Paola" last="Sandroni">Paola Sandroni</name>
<name sortKey="Tippmann Peikert, Maja" sort="Tippmann Peikert, Maja" uniqKey="Tippmann Peikert M" first="Maja" last="Tippmann-Peikert">Maja Tippmann-Peikert</name>
<name sortKey="Tobin, William Oliver" sort="Tobin, William Oliver" uniqKey="Tobin W" first="William Oliver" last="Tobin">William Oliver Tobin</name>
<name sortKey="Wirrell, Elaine C" sort="Wirrell, Elaine C" uniqKey="Wirrell E" first="Elaine C." last="Wirrell">Elaine C. Wirrell</name>
</noCountry>
</tree>
</affiliations>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Canada/explor/ParkinsonCanadaV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000B08 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 000B08 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Canada |area= ParkinsonCanadaV1 |flux= Main |étape= Exploration |type= RBID |clé= PMC:4240433 |texte= DPPX potassium channel antibody }}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Main/Exploration/RBID.i -Sk "pubmed:25320100" \ | HfdSelect -Kh $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd \ | NlmPubMed2Wicri -a ParkinsonCanadaV1
This area was generated with Dilib version V0.6.29. |