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Literature summary for 2.3.1.61 extracted from

  • Yang, L.; Shi, Q.; Ho, D.J.; Starkov, A.A.; Wille, E.J.; Xu, H.; Chen, H.L.; Zhang, S.; Stack, C.M.; Calingasan, N.Y.; Gibson, G.E.; Beal, M.F.
    Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins (2009), Neurobiol. Dis., 36, 320-330.
    View publication on PubMedView publication on EuropePMC

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
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Mus musculus 5739
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Organism

Organism UniProt Comment Textmining
Mus musculus
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wild-type and enzyme deficient mice (DLS+/-, C57BL/6, and 129SV/EV hybrid)
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Synonyms

Synonyms Comment Organism
dihydrolipoyl succinyl transferase
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Mus musculus
DLST
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Mus musculus

General Information

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