Immunoreactive Levels of α-ketoglutarate Dehydrogenase Subunits in Friedreich's Ataxia and Spinocerebellar Ataxia Type 1
✍ Scribed by Frank Mastrogiacomo; Jacques LaMarche; Slobodan Dožić; Gordon Lindsay; Lucien Bettendorff; Yves Robitaille; Lawrence Schut; Stephen J. Kish
- Book ID
- 115641875
- Publisher
- Elsevier Science
- Year
- 1996
- Tongue
- English
- Weight
- 113 KB
- Volume
- 5
- Category
- Article
- ISSN
- 1055-8330
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## Abstract Decreased blood and cerebrospinal fluid levels of thiamine have been reported in patients with spinocerebellar ataxia disorders. To determine whether a thiamine deficiency is present in the brain, we measured levels of thiamine and its phosphate esters thiamine monophosphate (TMP) and t
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 phosphatid
Much evidence, derived from biochemical studies of both blood and autopsied brain, has suggested that phospholipid metabolism is abnormal in patients with Friedreich's ataxia (FA), a disorder characterized by severe neuronal loss in the spinal cord and lower brain stem with no, or only modest, damag