Cytochrome P450 family 26 subfamily C member 1 (CYP26C1)

Cytochrome P450 family 26 subfamily C member 1 (CYP26C1) is a monooxygenase enzyme of the cytochrome P450 superfamily primarily responsible for the catabolism of retinoic acid (all-trans- and 9-cis-retinoic acid), thereby playing a critical role in the control of retinoic acid levels within tissues and cells[1][2][3]. This regulation is essential for diverse biological processes including embryonic development, neural patterning, and limb formation[3]. The enzyme oxidizes retinoic acid and its derivatives, limiting their biological activity and promoting their degradation[1]. Mutations or altered function in CYP26C1 can interact with other genetic factors such as SHOX gene mutations to modify the severity of clinical phenotypes related to skeletal growth and dysplasia[3]. CYP26C1 has been implicated as a genetic modifier in conditions such as SHOX deficiency, contributing to variation in disease expressivity. The enzyme belongs to a broader class of cytochrome P450s that are integral for xenobiotic metabolism, though CYP26C1 itself is more specialized for retinoid catabolism rather than broad drug metabolism. No specific, widely used drugs target CYP26C1 in clinical practice; however, its pathway is of interest for potential therapies in disorders of retinoic acid signaling. Manipulation of CYP26C1 activity poses risks related to inappropriate retinoic acid exposure, including teratogenesis and developmental anomalies[3].

Other names

Cytochrome P450 26C1CYP26C1FFDD4Cytochrome P450, family 26, subfamily C, polypeptide 1

Inhibition of retinoic acid oxidation (general for CYP26 family inhibitors); Regulation of intracellular retinoic acid levels