| Brain phospholipids and fatty acids in Friedreich's ataxia and spinocerebellar atrophy type-1. | |
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MedLine Citation:
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PMID: 9756151 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Previous studies of patients with spinocerebellar atrophy type 1 (SCA-1) and Friedreich's ataxia (FA) have suggested the occurrence of membrane disturbances in both disorders. We measured concentrations of phosphatidylcholine (PC), diacyl and plasmalogen phosphatidylethanolamine (PE), and phosphatidylserine (PS), along with their fatty acid profiles, in the brains of eight patients with Friedreich's ataxia (FA) and nine patients with dominantly inherited spinocerebellar atrophy type 1 (SCA-1). Compared with the controls, levels of all phospholipid types (PE, PS, and PC) were reduced in the cerebellar but not occipital cortex of SCA-1 patients. In contrast, in the FA group, levels of PS and PE, but not PC, were reduced in both cerebellar and occipital cortices. The fatty acid composition of individual brain phospholipids was altered in both FA and SCA-1 patients, most markedly in the plasmalogen PE and PS classes of cerebellar phospholipids. Given the neuropathologic characteristics of each disorder, it is likely that altered fatty acid composition and phospholipid levels in SCA-1 cerebellar cortex occur as a consequence of pronounced cerebellar degeneration. In contrast, reduced phospholipid levels in FA cerebellar and occipital cortex, areas characterized by, at most, minimal neuronal loss in FA, may represent a widespread alteration in cellular phospholipid metabolism occurring in response to the specific gene defect in the disorder. |
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Authors:
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K Eder; S J Kish; M Kirchgessner; B M Ross |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Movement disorders : official journal of the Movement Disorder Society Volume: 13 ISSN: 0885-3185 ISO Abbreviation: Mov. Disord. Publication Date: 1998 Sep |
Date Detail:
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Created Date: 1999-01-05 Completed Date: 1999-01-05 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 8610688 Medline TA: Mov Disord Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 813-9 Citation Subset: IM |
Affiliation:
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Institute of Nutrition Physiology, Technical University of Munich, Germany. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Atrophy Brain / pathology* Cerebellum / pathology Fatty Acids / metabolism* Female Friedreich Ataxia / diagnosis, genetics, pathology* Genes, Dominant Humans Male Middle Aged Occipital Lobe / pathology Phospholipids / metabolism* Pyramidal Tracts / pathology Spinocerebellar Degenerations / diagnosis, genetics, pathology* |
| Grant Support | |
ID/Acronym/Agency:
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NINDS 26034/DS/DS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Fatty Acids; 0/Phospholipids |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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