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== Function ==3-Ketoacyl-acyl carrier protein (ACP) reductase (KAR) catalyses the second step of the mtFAS pathway . Although all the other mtFAS enzymes identified thus far are encoded by single genes, human KAR (HsKAR) is a heterotetrametric α2β2 enzyme formed by two subunits, 17β-hydroxysteroid dehydrogenase type 8 (HSD17B8 or KE6, α-subunit) and carbonyl reductase type 4 (CBR4 or SDR45C1, β-subunit)9. HsHSD17B8 is expressed in abundance in the prostate, placenta and kidney, and has been shown to catalyse the reversible oxidation/reduction of steroid molecules in vitro. The physiological roles of HSD17B8 and CBR4 have remained elusive, and information is only available from studies of the respective homotetramers.
== Disease == There is evidence for physiologically relevant products of mtFAS in yeast6. Deficiency of mtFAS leads to respiratory chain defects and mitochondrial dysfunction
== Relevance == mtFAS provides the precursor of mitochondrially synthesized α-lipoic acid, a key cofactor for oxidative decarboxylation of α-keto acids and glycine within eukaryotic cells5. There is evidence for other physiologically relevant products of mtFAS in yeast6. Deficiency of mtFAS leads to respiratory chain defects and mitochondrial dysfunction
Structural highlights
is bound in each of the four subunits in heterotetrameric structure.
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