OGDH
| Identifiers | |
|---|---|
| EC number | 1.2.4.2 |
| CAS number | 9028-91-7 |
| Alt. names | |
| IntEnz | IntEnz view |
| BRENDA | BRENDA entry |
| ExPASy | NiceZyme view |
| KEGG | KEGG entry |
| MetaCyc | metabolic pathway |
2-oxoglutarate dehydrogenase (OGDH) is an important enzyme in the citric acid cycle (also known as the Krebs cycle or TCA cycle). It catalyzes the conversion of 2-oxoglutarate (also known as alpha-ketoglutarate) into succinyl-CoA and carbon dioxide. This reaction is a key step in the metabolic pathway that generates adenosine triphosphate (ATP) through the oxidation of acetyl-CoA.
Function[edit | edit source]
OGDH is a component of the 2-oxoglutarate dehydrogenase complex, which is a multi-enzyme complex that also includes dihydrolipoamide succinyltransferase and dihydrolipoamide dehydrogenase. This complex is responsible for the decarboxylation of 2-oxoglutarate, a critical step in the citric acid cycle.
Structure[edit | edit source]
The enzyme is a large, multi-subunit complex that requires several cofactors for its activity, including thiamine pyrophosphate (TPP), lipoic acid, coenzyme A (CoA), FAD, and NAD+.
Clinical significance[edit | edit source]
Mutations or deficiencies in OGDH can lead to metabolic disorders and have been associated with neurological diseases. The enzyme's activity is crucial for maintaining normal cellular energy levels, and its dysfunction can result in mitochondrial diseases.
Regulation[edit | edit source]
OGDH activity is regulated by the availability of its substrates and cofactors, as well as by feedback inhibition from its products, such as NADH and succinyl-CoA.
See also[edit | edit source]
References[edit | edit source]
External links[edit | edit source]
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