Cerebellar alpha-ketoglutarate dehydrogenase activity is reduced in spinocerebellar ataxia type 1
| Autor: | Stephen J. Kish, Frank Mastrogiacomo |
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| Rok vydání: | 1994 |
| Předmět: |
Adult
medicine.medical_specialty Cerebellum Spinocerebellar Ataxia Type 1 Ataxia Glutamic Acid Biology chemistry.chemical_compound Olivopontocerebellar atrophy Glutamates Reference Values Internal medicine medicine Humans Ketoglutarate Dehydrogenase Complex Spinocerebellar Degenerations Cerebral Cortex Aspartic Acid Neurotransmitter Agents medicine.disease Citric acid cycle medicine.anatomical_structure Endocrinology Neurology chemistry Biochemistry Cerebellar cortex Thiamine Neurology (clinical) medicine.symptom Energy Metabolism Thiamine pyrophosphate |
| Zdroj: | Annals of neurology. 35(5) |
| ISSN: | 0364-5134 |
| Popis: | We measured the activity of the thiamine pyrophosphate-dependent enzyme alpha-ketoglutarate dehydrogenase complex in postmortem brain of 12 patients with the spinocerebellar ataxia type 1 form of olivopontocerebellar atrophy. alpha-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. |
| Databáze: | OpenAIRE |
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