Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Mus musculus | 5739 | - |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | - |
wild-type and enzyme deficient mice (DLS+/-, C57BL/6, and 129SV/EV hybrid) | - |
Synonyms | Comment | Organism |
---|---|---|
dihydrolipoyl succinyl transferase | - |
Mus musculus |
DLST | - |
Mus musculus |
General Information | Comment | Organism |
---|---|---|
malfunction | enzyme deficient mice show reduced mRNA and protein levels and decreased brain mitochondrial alpha-ketoglutarate dehydrogenase activity (by about 40%), increased vulnerability to mitochondrial toxins: MPTP treatment enhances the severity of lipid peroxidation in the substantial nigra, reduced striatal dopamine (59% depletion in wild-type, 73% in enzyme deficient mutants), dopaminergic neurons (25% reduction in wild-type, 42% in mutants) and tyrosine hydroxylase-positive neurons, striatal lesions induced by malonate (mimicking Huntington's disease, 2fold larger lesions, smaller striatum) or 3-nitropropionic acid (5fold larger lesions than in wild-type) are significantly larger in enzyme deficient mice than in the wild-type, and the 3-nitropropionic acid-induced mitochondrial enzyme inhibition (25% lower citrate synthase activity than in wild-type), protein and DNA oxidation is enhanced in the cortex of enzyme deficient mice compared to wild-type | Mus musculus |
physiological function | subunit of the alpha-ketoglutarate dehydrogenase complex | Mus musculus |