3-hydroxy-2-methylbutyryl-CoA dehydrogenase
| 3-hydroxy-2-methylbutyryl-CoA dehydrogenase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC number | 1.1.1.178 | ||||||||
| CAS number | 37250-24-1 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
| |||||||||
3-hydroxy-2-methylbutyryl-CoA dehydrogenase is an enzyme that plays a crucial role in the isoleucine degradation pathway. It catalyzes the conversion of 3-hydroxy-2-methylbutyryl-CoA to 2-methylacetoacetyl-CoA, utilizing NAD+ as a cofactor and producing NADH in the process.
Function[edit | edit source]
This enzyme is involved in the catabolism of the branched-chain amino acid isoleucine. The reaction it catalyzes is a part of the metabolic pathway that breaks down isoleucine into acetyl-CoA and propionyl-CoA, which can then enter the citric acid cycle or be used in other metabolic processes.
Reaction[edit | edit source]
The chemical reaction catalyzed by 3-hydroxy-2-methylbutyryl-CoA dehydrogenase is as follows:
- 3-hydroxy-2-methylbutyryl-CoA + NAD+ → 2-methylacetoacetyl-CoA + NADH + H+
This reaction is an oxidation-reduction process where NAD+ is reduced to NADH, and the substrate 3-hydroxy-2-methylbutyryl-CoA is oxidized to 2-methylacetoacetyl-CoA.
Structure[edit | edit source]
The enzyme is a member of the short-chain dehydrogenase/reductase (SDR) family. It typically functions as a homotetramer, with each subunit contributing to the active site. The structure of the enzyme allows it to bind both the substrate and the cofactor NAD+ effectively, facilitating the transfer of electrons.
Clinical Significance[edit | edit source]
Deficiencies in the activity of 3-hydroxy-2-methylbutyryl-CoA dehydrogenase can lead to metabolic disorders related to isoleucine metabolism. Such deficiencies may result in the accumulation of toxic intermediates, which can cause symptoms ranging from mild to severe metabolic acidosis.
Also see[edit | edit source]
References[edit | edit source]
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Contributors: Prab R. Tumpati, MD
