Human Keto Acyl Reductase: Difference between revisions
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<StructureSection load='4cqm' size='240' side='right' caption='Human ketoacyl reductase complex with NADP (PDB code [[4cqm]])' scene=''> | <StructureSection load='4cqm' size='240' side='right' caption='Human ketoacyl reductase complex with NADP (PDB code [[4cqm]])' scene=''> | ||
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==Function== | ==Function== | ||
The mitochondrial fatty acid synthesis (mtFAS) pathway generates precursors for mitochondrally generated alpha-lipoic acids. Defeciency of mtFAS leads to respiratory chain degects and mitochondrial dysfunction. The mtFAS has four enzymatic steps, catalyzed by four different enzymes. Ketoacyl acyl-carrier-protein (ACP) reductase (KAR) catalyzes the second step of mtFAS pathway, where 3-ketoacyl-ACP is reduced to 3R-hydroxyacyl-ACP utilizing NADPH as cofactor. KAR is a heterotetrameric complex made from two different polypeptides: 17β-hydroxysteroid dehydrogenase type 8 (HSD17B8 or KE6, α-subunit) and carbonyl reductase type 4 (CBR4 or SDR45C1, β-subunit)9. <ref>PMID:25203508</ref> | The mitochondrial fatty acid synthesis (mtFAS) pathway generates precursors for mitochondrally generated alpha-lipoic acids. Defeciency of mtFAS leads to respiratory chain degects and mitochondrial dysfunction. The mtFAS has four enzymatic steps, catalyzed by four different enzymes. Ketoacyl acyl-carrier-protein (ACP) reductase (KAR) catalyzes the second step of mtFAS pathway, where 3-ketoacyl-ACP is reduced to 3R-hydroxyacyl-ACP utilizing NADPH as cofactor. KAR is a heterotetrameric complex made from two different polypeptides: 17β-hydroxysteroid dehydrogenase type 8 (HSD17B8 or KE6, α-subunit) and carbonyl reductase type 4 (CBR4 or SDR45C1, β-subunit)9. <ref>PMID:25203508</ref> | ||
==Disease | ==Disease== | ||
Deficiency of the KAR or mtFAS pathway leads to respiratory chain defects and mitochondrial dysfunction in eukaryotes. | Deficiency of the KAR or mtFAS pathway leads to respiratory chain defects and mitochondrial dysfunction in eukaryotes. | ||
