Function of CYP11A1 in the mitochondria.

Steroids are synthesized from the adrenal glands and gonads by enzymes of the cytochromes P450 and hydroxysteroid dehydrogenase in nature. These enzymes are located in the membrane of endoplasmic reticulum and mitochondria to catalyze redox reactions using electrons transported from the membrane. In the mitochondria, steroidogenic enzymes are inserted into the inner membrane with the bulk of the protein facing the matrix. They are not only important for steroid biosynthesis, their presence also affects mitochondrial morphology. Mitochondria undergo constant fission and fusion; they play important roles in energy production, apoptosis, and metabolism. Their defects often lead to human diseases. Mitochondrial cristae are usually lamellar in shape, but can also assume different shapes. Cristae in the mitochondria of steroidogenic cells are tubular-vesicular in shape. This cristae shape is also related to the degree of steroidogenic cell differentiation. Steroidogenic enzymes in the mitochondria appear to have a dual role in shaping the morphology of mitochondria and in steroid production.