Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease
Identifieur interne : 006653 ( Main/Merge ); précédent : 006652; suivant : 006654Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease
Auteurs : Eric K. Richfield [États-Unis] ; Jean-Paul Vonsattel [États-Unis] ; Marcy E. Macdonald [États-Unis] ; ZHIQIANG SUN [États-Unis] ; Yun-Ping P. Deng [États-Unis] ; Anton Reiner [États-Unis]Source :
- Movement disorders [ 0885-3185 ] ; 2002.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
Abstract
Phenocopies of Huntington's disease (HD) are individuals with a family history, clinical symptoms, and occasionally pathological evidence of HD but without an expanded CAG repeat within the HD gene. We report on an HD phenocopy with selective loss of preprotachykinin (PPT) neurons, dysfunction of surviving PPT neurons, preservation of preproenkephalin (PPE) neurons within the striatum, and greater loss of immunohistochemical staining for substance P in terminals of striatal neurons projecting to the substantia nigra, than in those projecting to the internal pallidal segment. This case demonstrates the existence of one type of striatal lesion that may produce a clinical picture similar to HD, and raises the possibility of a rare hereditary disease that mimics HD.
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Pascal:02-0309341Le document en format XML
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Richfield</name><affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Department of Pathology and Laboratory Medicine and Center for Aging and Developmental Biology, University of Rochester School of Medicine and Dentistry</s1><s2>Rochester, New York</s2><s3>USA</s3><sZ>1 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">État de New York</region></placeName></affiliation></author><author><name sortKey="Vonsattel, Jean Paul" sort="Vonsattel, Jean Paul" uniqKey="Vonsattel J" first="Jean-Paul" last="Vonsattel">Jean-Paul Vonsattel</name><affiliation wicri:level="2"><inist:fA14 i1="02"><s1>Laboratory for Molecular Neuropathology, Massachusetts General Hospital</s1><s2>Charlestown, Massachusetts</s2><s3>USA</s3><sZ>2 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Massachusetts</region></placeName></affiliation></author><author><name sortKey="Macdonald, Marcy E" sort="Macdonald, Marcy E" uniqKey="Macdonald M" first="Marcy E." last="Macdonald">Marcy E. 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Richfield</name><affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Department of Pathology and Laboratory Medicine and Center for Aging and Developmental Biology, University of Rochester School of Medicine and Dentistry</s1><s2>Rochester, New York</s2><s3>USA</s3><sZ>1 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">État de New York</region></placeName></affiliation></author><author><name sortKey="Vonsattel, Jean Paul" sort="Vonsattel, Jean Paul" uniqKey="Vonsattel J" first="Jean-Paul" last="Vonsattel">Jean-Paul Vonsattel</name><affiliation wicri:level="2"><inist:fA14 i1="02"><s1>Laboratory for Molecular Neuropathology, Massachusetts General Hospital</s1><s2>Charlestown, Massachusetts</s2><s3>USA</s3><sZ>2 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Massachusetts</region></placeName></affiliation></author><author><name sortKey="Macdonald, Marcy E" sort="Macdonald, Marcy E" uniqKey="Macdonald M" first="Marcy E." last="Macdonald">Marcy E. Macdonald</name><affiliation wicri:level="2"><inist:fA14 i1="03"><s1>Molecular Neurogenetics Unit, Massachusetts General Hospital</s1><s2>Charlestown, Massachusetts</s2><s3>USA</s3><sZ>3 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Massachusetts</region></placeName></affiliation></author><author><name sortKey="Zhiqiang Sun" sort="Zhiqiang Sun" uniqKey="Zhiqiang Sun" last="Zhiqiang Sun">ZHIQIANG SUN</name><affiliation wicri:level="2"><inist:fA14 i1="04"><s1>Department of Anatomy and Neurobiology, University of Tennessee, Memphis</s1><s2>Memphis, Tennessee</s2><s3>USA</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Tennessee</region></placeName></affiliation></author><author><name sortKey="Deng, Yun Ping P" sort="Deng, Yun Ping P" uniqKey="Deng Y" first="Yun-Ping P." last="Deng">Yun-Ping P. Deng</name><affiliation wicri:level="2"><inist:fA14 i1="04"><s1>Department of Anatomy and Neurobiology, University of Tennessee, Memphis</s1><s2>Memphis, Tennessee</s2><s3>USA</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Tennessee</region></placeName></affiliation></author><author><name sortKey="Reiner, Anton" sort="Reiner, Anton" uniqKey="Reiner A" first="Anton" last="Reiner">Anton Reiner</name><affiliation wicri:level="2"><inist:fA14 i1="04"><s1>Department of Anatomy and Neurobiology, University of Tennessee, Memphis</s1><s2>Memphis, Tennessee</s2><s3>USA</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Tennessee</region></placeName></affiliation></author></analytic><series><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno><imprint><date when="2002">2002</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Corpus striatum</term><term>Human</term><term>Huntington disease</term><term>Immunohistochemistry</term><term>Loss</term><term>Neuron</term><term>Pathophysiology</term><term>Substance P</term><term>Tachykinin</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Chorée Huntington</term><term>Immunohistochimie</term><term>Tachykinine</term><term>Corps strié</term><term>Perte</term><term>Neurone</term><term>Substance P</term><term>Physiopathologie</term><term>Homme</term><term>Préprotachykinine</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Phenocopies of Huntington's disease (HD) are individuals with a family history, clinical symptoms, and occasionally pathological evidence of HD but without an expanded CAG repeat within the HD gene. We report on an HD phenocopy with selective loss of preprotachykinin (PPT) neurons, dysfunction of surviving PPT neurons, preservation of preproenkephalin (PPE) neurons within the striatum, and greater loss of immunohistochemical staining for substance P in terminals of striatal neurons projecting to the substantia nigra, than in those projecting to the internal pallidal segment. This case demonstrates the existence of one type of striatal lesion that may produce a clinical picture similar to HD, and raises the possibility of a rare hereditary disease that mimics HD.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Massachusetts</li><li>Tennessee</li><li>État de New York</li></region></list><tree><country name="États-Unis"><region name="État de New York"><name sortKey="Richfield, Eric K" sort="Richfield, Eric K" uniqKey="Richfield E" first="Eric K." last="Richfield">Eric K. Richfield</name></region><name sortKey="Deng, Yun Ping P" sort="Deng, Yun Ping P" uniqKey="Deng Y" first="Yun-Ping P." last="Deng">Yun-Ping P. Deng</name><name sortKey="Macdonald, Marcy E" sort="Macdonald, Marcy E" uniqKey="Macdonald M" first="Marcy E." last="Macdonald">Marcy E. Macdonald</name><name sortKey="Reiner, Anton" sort="Reiner, Anton" uniqKey="Reiner A" first="Anton" last="Reiner">Anton Reiner</name><name sortKey="Vonsattel, Jean Paul" sort="Vonsattel, Jean Paul" uniqKey="Vonsattel J" first="Jean-Paul" last="Vonsattel">Jean-Paul Vonsattel</name><name sortKey="Zhiqiang Sun" sort="Zhiqiang Sun" uniqKey="Zhiqiang Sun" last="Zhiqiang Sun">ZHIQIANG SUN</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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