功能： catalytic activity:3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholanoyl-CoA + propanoyl-CoA = CoA + 3-alpha,7-alpha,12-alpha-trihydroxy-24-oxo-5-beta-cholestanoyl-CoA.,caution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,disease:SCP2 is present in low levels in subjects with Zellweger syndrome (cerebro-hepatic-renal syndrome), whose cells are deficient in peroxisomes and who have an associated impairment in plasmalogen and bile acid synthesis and catabolism of phytanic acid and very-long-chain fatty acids.,function:Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis.,similarity:Belongs to the thiolase family.,similarity:Contains 1 SCP2 domain.,similarity:In the N-terminal section; belongs to the thiolase family.,subcellular location:Cytoplasmic in the liver and also associated with mitochondria especially in steroidogenic tissues.,subcellular location:Interaction with PEX5 is essential for peroxisomal import.,subunit:Interacts with PEX5.,tissue specificity:Liver, fibroblasts, and placenta.,
功能： catalytic activity:3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholanoyl-CoA + propanoyl-CoA = CoA + 3-alpha,7-alpha,12-alpha-trihydroxy-24-oxo-5-beta-cholestanoyl-CoA.,caution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,disease:SCP2 is present in low levels in subjects with Zellweger syndrome (cerebro-hepatic-renal syndrome), whose cells are deficient in peroxisomes and who have an associated impairment in plasmalogen and bile acid synthesis and catabolism of phytanic acid and very-long-chain fatty acids.,function:Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis.,similarity:Belongs to the thiolase family.,similarity:Contains 1 SCP2 domain.,similarity:In the N-terminal section; belongs to the thiolase family.,subcellular location:Cytoplasmic in the liver and also associated with mitochondria especially in steroidogenic tissues.,subcellular location:Interaction with PEX5 is essential for peroxisomal import.,subunit:Interacts with PEX5.,tissue specificity:Liver, fibroblasts, and placenta.,
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