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The α‐Ketoglutarate Dehydrogenase Complex

Annals of the New York Academy of Sciences, 1999
ABSTRACT: The α‐ketoglutarate dehydrogenase complex (KGDHC) is an important mitochondrial constituent, and deficiency of KGDHC is associated with a number of neurological disorders. KGDHC is composed of three proteins, each encoded on a different and well‐characterized gene. The sequences of the human proteins are known.
KWAN‐FU REX SHEU, JOHN P. BLASS
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The α-ketoglutarate dehydrogenase complex in neurodegeneration

Neurochemistry International, 2000
Altered energy metabolism is characteristic of many neurodegenerative disorders. Reductions in the key mitochondrial enzyme complex, the alpha-ketoglutarate dehydrogenase complex (KGDHC), occur in a number of neurodegenerative disorders including Alzheimer's Disease (AD).
G E, Gibson   +4 more
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Secondary inhibition of 2-ketoglutarate dehydrogenase complex by MPTP

NeuroReport, 1998
The parkinsonism-inducing neurotoxin 1-methyl-4-phenylpyridine (MPP+) acts through inhibition of complex I of the electron transport chain. Recent evidence suggests that it may also act through inhibition of 2-ketoglutarate dehydrogenase complex (KDHC).
G T, Joffe, J K, Parks, W D, Parker
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α-Ketoglutarate dehydrogenase complex may be heterogeneous in quaternary structure

Journal of Molecular Biology, 1983
The quaternary structure of the alpha-ketoglutarate dehydrogenase complex (KGDC) from Escherichia coli has been investigated by electron microscopy. KGDC consists of an octahedral cube-shaped structural core, lipoyl transsuccinylase (E2), to which 12 polypeptide chains each of alpha-ketoglutarate dehydrogenase (E1) and dihydrolipoyl dehydrogenase (E3 ...
T, Wagenknecht   +2 more
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Association between the α-ketoglutarate dehydrogenase complex and succinate thiokinase

Biochimica et Biophysica Acta (BBA) - Protein Structure and Molecular Enzymology, 1983
The kinetic parameters of the individual reaction of pig heart alpha-ketoglutarate dehydrogenase complex, succinate thiokinase and the alpha-ketoglutarate dehydrogenase complex-succinate thiokinase coupled system were studied. The KCoAm of alpha-ketoglutarate dehydrogenase complex and the K-succinyl CoAm of succinate thiokinase decreased in the coupled
Z, Porpáczy, B, Sümegi, I, Alkonyi
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The composition of the ketoglutarate dehydrogenase complex

Biochimica et Biophysica Acta, 1960
Abstract Ketoglutarate dehydrogenase has been isolated from pig heart as a soluble complex of enzymes, containing protein bound thiamin pyrophosphate, lipoic acid and FAD in stoichiometric amounts. The flavin constituent of the complex has been shown to be lipoyl dehydrogenase.
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Distribution of the α‐ketoglutarate dehydrogenase complex in rat brain

Journal of Comparative Neurology, 1994
AbstractThe α‐ketoglutarate dehydrogenase complex (KGDHC) is a key enzyme in mitochondrial oxidation that appears critical to neurodegenerative diseases. Its activity in the brain declines in thiamine‐deficient animals, Alzheimer's disease, and Wernicke‐Korsakoff syndrome.
N Y, Calingasan   +3 more
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Structure-Function Relationships in Pyruvate and α-Ketoglutarate Dehydrogenase Complexes

1982
Enzyme systems that catalyze a lipoic acid-mediated oxidative decarboxylation of pyruvate and α-ketoglutarate have been isolated from microbial and eukaryotic cells as functional units with molecular weights in the millions. Each complex consists of three catalytic components: pyruvate dehydrogenase or α -ketoglutarate dehydrogenase (E1); dihydrolipoyl
L J, Reed, R M, Oliver
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