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Present mainly in the mitochondrial compartment of peripheral tissues, the protein encoded by this gene interacts with some benzodiazepines and has different affinities than its endogenous counterpart. The protein is a key factor in the flow of cholesterol into mitochondria to permit the initiation of steroid hormone synthesis. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

Gene ID: 7428
elongin binding protein , HRCA1 , Protein G7 , pVHL , RCA1 , VHL1 , von Hippel-Lindau disease tumor suppressor , von Hippel-Lindau syndrome , von Hippel-Lindau tumor suppressor