Brain phospholipids and fatty acids in Friedreich's ataxia and spinocerebellar atrophy type‐1
Identifieur interne : 005061 ( Main/Exploration ); précédent : 005060; suivant : 005062Brain phospholipids and fatty acids in Friedreich's ataxia and spinocerebellar atrophy type‐1
Auteurs : Klaus Eder [Allemagne] ; Stephen J. Kish [Canada] ; Manfred Kirchgessner [Allemagne] ; Ross [Canada]Source :
- Movement Disorders [ 0885-3185 ] ; 1998-09.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
- Adult, Atrophy, Brain (pathology), Brain (vertebrata), Cerebellum, Cerebellum (pathology), Exploration, Fatty Acids (metabolism), Fatty acids, Female, Friedreich Ataxia (diagnosis), Friedreich Ataxia (genetics), Friedreich Ataxia (pathology), Friedreich ataxia, Genes, Dominant, Human, Humans, Male, Membrance, Middle Aged, Occipital Lobe (pathology), Occipital cortex, Phospholipid, Phospholipids (metabolism), Plasmalogen, Pyramidal Tracts (pathology), Spinocerebellar Degenerations (diagnosis), Spinocerebellar Degenerations (genetics), Spinocerebellar Degenerations (pathology).
- MESH :
- chemical , metabolism : Fatty Acids, Phospholipids.
- diagnosis : Friedreich Ataxia, Spinocerebellar Degenerations.
- genetics : Friedreich Ataxia, Spinocerebellar Degenerations.
- pathology : Brain, Cerebellum, Friedreich Ataxia, Occipital Lobe, Pyramidal Tracts, Spinocerebellar Degenerations.
- Adult, Atrophy, Female, Genes, Dominant, Humans, Male, Middle Aged.
Abstract
Previous studies of patients with spinocerebellar atrophy type 1 (SCA‐1) and Friedreich's ataxia (FA) have suggested the occurrence of membrane distrubances 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.
Url:
DOI: 10.1002/mds.870130510
Affiliations:
- Allemagne, Canada
- Bavière, District de Haute-Bavière, Ontario
- Munich, Toronto
- Université Louis-et-Maximilien de Munich, Université de Toronto
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Le document en format XML
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<term>Brain (vertebrata)</term>
<term>Cerebellum</term>
<term>Cerebellum (pathology)</term>
<term>Exploration</term>
<term>Fatty Acids (metabolism)</term>
<term>Fatty acids</term>
<term>Female</term>
<term>Friedreich Ataxia (diagnosis)</term>
<term>Friedreich Ataxia (genetics)</term>
<term>Friedreich Ataxia (pathology)</term>
<term>Friedreich ataxia</term>
<term>Genes, Dominant</term>
<term>Human</term>
<term>Humans</term>
<term>Male</term>
<term>Membrance</term>
<term>Middle Aged</term>
<term>Occipital Lobe (pathology)</term>
<term>Occipital cortex</term>
<term>Phospholipid</term>
<term>Phospholipids (metabolism)</term>
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<term>Pyramidal Tracts (pathology)</term>
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<term>Spinocerebellar Degenerations (genetics)</term>
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<front><div type="abstract" xml:lang="en">Previous studies of patients with spinocerebellar atrophy type 1 (SCA‐1) and Friedreich's ataxia (FA) have suggested the occurrence of membrane distrubances 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.</div>
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