Mitochondrial enzymes in hereditary ataxias
Sheu K, Blass J, Cedarbaum J, Kim Y, Harding B, DeCicco J. Mitochondrial enzymes in hereditary ataxias. Metabolic Brain Disease 1988, 3: 151-160. PMID: 3185426, DOI: 10.1007/bf01001015.Peer-Reviewed Original ResearchConceptsHereditary ataxiasUnselected seriesCross-reacting materialSecondary decreasePatientsMitochondrial abnormalitiesAtaxiaPyruvate dehydrogenase complexMitochondrial damageMitochondrial enzymesPlateletsNormal fibroblastsGlutamate dehydrogenaseHuman brainAtaxyBrainImmunoblotFibroblastsPathophysiologyActivityAbnormalities