1.2.1.105: 2-oxoglutarate dehydrogenase system
This is an abbreviated version!
For detailed information about 2-oxoglutarate dehydrogenase system, go to the full flat file.
Word Map on EC 1.2.1.105
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1.2.1.105
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kgdhc
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dihydrolipoamide
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thiamin
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alpha-ketoacids
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2-oxoadipate
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transsuccinylase
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charcot-marie-tooth
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2-aminoadipic
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medicine
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thiokinase
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glutaryl-coa
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analysis
- 1.2.1.105
- kgdhc
- dihydrolipoamide
- thiamin
- alpha-ketoacids
- 2-oxoadipate
-
transsuccinylase
- charcot-marie-tooth
-
2-aminoadipic
- medicine
- thiokinase
- glutaryl-coa
- analysis
Reaction
Synonyms
2-OGDH2, 2-oxoglutarate dehydrogenase, 2-oxoglutarate dehydrogenase complex, alpha-KDE2, alpha-ketoglutarate dehydrogenase, alpha-ketoglutarate dehydrogenase complex, alpha-KGDH, At3g55410, At5g65750, DHTKD1, dihydrolipoyl succinyltransferase E2, E1a, E1k, E1o, E2, KGDH, KGDHC, More, MPA24.10, ODGH, ODGH1, ODGH2, ODH, OGDC, OGDH, OGDHC, OGDHL, OGHDC-E2, PDHC, SucA, SucB
ECTree
1.2.1.105 2-oxoglutarate dehydrogenase systemAdvanced search results
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results (Natural Substrates Products
Natural Substrates Products on EC 1.2.1.105 - 2-oxoglutarate dehydrogenase system
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NATURAL SUBSTRATE 
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
2-oxoglutarate + lipoamide
S-succinyldihydrolipoamide + CO2
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the 2-oxoglutarate dehydrogenase complex catalyzes a critical step in the Krebs tricarboxylic acid cycle, which is also a step in the metabolism of the potentially excitotoxic neurotransmitter glutamate. Deficiencies of the 2-oxoglutarate dehydrogenase complex are likely to impair energy metabolism and therfore brain function, and lead to manifestations of brain disease. Neurons that are enriched in the 2-oxoglutarate dehydrogenase complex may be selectively vulnerable in Alzheimers disease. Variations in 2-oxoglutarate dehydrogenase complex that are not deleterious during reproductive life become deleterious with aging, perhaps by predisposing this mitochondrial metabolon to oxidative damage
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?
2-oxoglutarate + lipoamide
S-succinyldihydrolipoamide + CO2
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reductions in enzyme activity occurs in a number of neurodegenerative disorders including Alzheimers disease. The reduction in 2-oxoglutarate dehydrogenase complex activity can be linked to several aspects of brain dysfunction and neuropathology in a number of neurodegenerative diseases
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?
2-oxoglutarate + lipoamide
S-succinyldihydrolipoamide + CO2
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catalyzes a rate limiting step of the TCA cycle
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?
2-oxoglutarate + lipoamide
S-succinyldihydrolipoamide + CO2
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?
2-oxoglutarate + lipoamide
S-succinyldihydrolipoamide + CO2
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enzyme of the TCA cycle
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?
2-oxoglutarate + lipoamide
S-succinyldihydrolipoamide + CO2
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addition of glutamate stimulates the synthesis of the 2-oxoglutarate dehydrogenase complex
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?
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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-
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ir
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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-
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ir
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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-
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ir
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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-
-
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ir
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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-
-
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ir
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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enzyme E2 is a component of the 2-oxoglutarate dehydrogenase multienzyme complex, rate-limiting enzyme in mitochondrial Krebs cycle
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ir
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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key step, probably rate-limiting, in the tricarboxylic acid cycle, physiologic function and regulation
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ir
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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-
-
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ir
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
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ir
additional information
?
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substrate channeling in the 2-oxo acid dehydrogenase multienzyme complex, mechanism, overview
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?
additional information
?
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substrate channeling in the 2-oxo acid dehydrogenase multienzyme complex, mechanism, overview
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?
additional information
?
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substrate channeling in the 2-oxo acid dehydrogenase multienzyme complex, mechanism, overview
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?
additional information
?
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enzyme activity is reduced in patients with Parkinson's disease due to elevated levels of monoamine oxidase B, physiological effects and regulation, overview
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?
additional information
?
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loss of enzyme activity contributes to the major loss of cerebral energy metabolism leading to a number of neurodegenerative disorders and Alzheimer's disease, myeloperoxidase activity is involved
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?
additional information
?
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reduced enzyme activity in vivo does not lead to the Ca2+ abnormalities observed in patients suffering brain disorders and abnormal mitochondrial function, or Alzheimer's disease, but the release of cytochrome c together with reduced enzyme activity activates other pathways including cell death cascades, enzyme inhibition alters Ca2+ homeostasis and increases cytosolic accumulation of cytochrome c
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?
additional information
?
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the enzyme is targeted for ubiquitination-dependent degradation in mitochondria by binding of Siah2, the RING finger ubiquitin-protein isopeptide ligase 2, encoded by gene siah2
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?
additional information
?
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substrate channeling in the 2-oxo acid dehydrogenase multienzyme complex, mechanism, overview
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?
additional information
?
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2-oxoglutarate-supported mitochondrial respiration state 3
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?
additional information
?
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KGDHC contributes to neuronal reactive oxygen species increase in situ
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?
additional information
?
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the KGDH enzyme has also a function as a poly(ADP-ribose) polymerase-like enzyme, which may play a role in regulating intramitochondrial NAD+ and poly(ADP-ribose) homeostasis
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?
