Article
Conditional deletion of cytochrome p450 reductase in osteoprogenitor cells affects long bone and skull development in mice recapitulating antley-bixler syndrome: role of a redox enzyme in development.
PloS one - 1 Jan 2013
Panda Satya P, Guntur Anyonya R, Polusani Srikanth R, Fajardo Roberto J, Gakunga Peter T, Roman Linda J, Masters Bettie Sue
Abstract excerpt
NADPH-cytochrome P450 oxidoreductase (POR) is the primary electron donor for cytochromes P450, dehydrocholesterol reductase, heme oxygenase, and squalene monooxygenase. Human patients with specific mutations in POR exhibit severe developmental malformations including disordered steroidogenesis, sexual ambiguities and various bone defects, similar to those seen in patients with Antley-Bixler syndrome (ABS). To...
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