Human Keto Acyl Reductase: Difference between revisions
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You may include any references to papers as in: the use of JSmol in Proteopedia <ref>DOI 10.1002/ijch.201300024</ref> or to the article describing Jmol <ref>PMID:21638687</ref> to the rescue. | You may include any references to papers as in: the use of JSmol in Proteopedia <ref>DOI 10.1002/ijch.201300024</ref> or to the article describing Jmol <ref>PMID:21638687</ref> to the rescue. | ||
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> | |||
== Disease == Deficiency of the KAR or mtFAS pathway leads to respiratory chain defects and mitochondrial dysfunction in eukaryotes. | == Disease == Deficiency of the KAR or mtFAS pathway leads to respiratory chain defects and mitochondrial dysfunction in eukaryotes. | ||
