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Cerebellar α‐Ketoglutarate dehydrogenase activity is reduced in spinocerebellar ataxia type 1
Author(s) -
Mastrogiacomo Frank,
Kish Stephen J.
Publication year - 1994
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410350519
Subject(s) - olivopontocerebellar atrophy , thiamine pyrophosphate , thiamine , ataxia , chemistry , cerebellar ataxia , pyrophosphate , cerebellum , citric acid cycle , biochemistry , enzyme , endocrinology , medicine , neuroscience , biology , degenerative disease , central nervous system disease , cofactor
We measured the activity of the thiamine pyrophosphate–dependent enzyme α‐ketoglutarate dehydrogenase complex in postmortem brain of 12 patients with the spinocerebellar ataxia type 1 form of olivopontocerebellar atrophy. α‐Ketoglutarate dehydrogenase complex activity measured in the absence of thiamine pyrophosphate was markedly reduced (−72%) in olivopontocerebellar atrophy cerebellar cortex. Decreased activity of this key rate‐limiting Krebs cycle enzyme could compromise cerebellar energy metabolism and excitatory amino acid synthesis and thereby contribute to the brain dysfunction of olivopontocerebellar atrophy.