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23 ABCG5 and ABCG8 genes in the STSL locus on chromosome 2p21 encode proteins sterolin-1 and sterolin-2, respectively, which together form a heterodimer pair expressed on the canalicular hepatocyte membrane and are involved in the transport of hepatobiliary cholesterol from hepatocytes into the bile canaliculi
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This results in the accumulation of electrons which can trigger ROS generation, as well as an accumulation of acyl-CoA which further disrupts mitochondrial function and signaling
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