特异性： This antibody detects endogenous levels of human AMACR. Heat-induced epitope retrieval (HIER) TRIS-EDTA of pH8.0 was highly recommended as antigen repair method in paraffin section
组成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源： Mouse, Monoclonal/IgG1, Kappa
稀释： IHC-p 1:200-400,WB 1:500-2000
纯化工艺： The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
储存： -15°C to -25°C/1 year(Do not lower than -25°C)
功能： catalytic activity:(2S)-2-methylacyl-CoA = (2R)-2-methylacyl-CoA.,disease:Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:604489]. AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. It can be associated with polyneuropathy, retinitis pigmentosa, epilepsy.,disease:Defects in AMACR are the cause of congenital bile acid synthesis defect type 4 (CBAS4) [MIM:214950]; also known as cholestasis, intrahepatic, with defective conversion of trihydroxycoprostanic acid to cholic acid or trihydroxycoprostanic acid in bile. Clinical features include neonatal jaundice, intrahepatic cholestasis, bile duct deficiency and absence of cholic acid from bile.,function:Racemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers.,pathway:Lipid metabolism; bile acid biosynthesis.,pathway:Lipid metabolism; fatty acid metabolism.,similarity:Belongs to the caiB/baiF CoA-transferase family.,similarity:Contains 1 C1q domain.,similarity:Contains 1 collagen-like domain.,
特异性： This antibody detects endogenous levels of human AMACR. Heat-induced epitope retrieval (HIER) TRIS-EDTA of pH8.0 was highly recommended as antigen repair method in paraffin section
组成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源： Mouse, Monoclonal/IgG1, Kappa
稀释： IHC-p 1:200-400,WB 1:500-2000
纯化工艺： The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
储存： -15°C to -25°C/1 year(Do not lower than -25°C)
功能： catalytic activity:(2S)-2-methylacyl-CoA = (2R)-2-methylacyl-CoA.,disease:Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:604489]. AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. It can be associated with polyneuropathy, retinitis pigmentosa, epilepsy.,disease:Defects in AMACR are the cause of congenital bile acid synthesis defect type 4 (CBAS4) [MIM:214950]; also known as cholestasis, intrahepatic, with defective conversion of trihydroxycoprostanic acid to cholic acid or trihydroxycoprostanic acid in bile. Clinical features include neonatal jaundice, intrahepatic cholestasis, bile duct deficiency and absence of cholic acid from bile.,function:Racemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers.,pathway:Lipid metabolism; bile acid biosynthesis.,pathway:Lipid metabolism; fatty acid metabolism.,similarity:Belongs to the caiB/baiF CoA-transferase family.,similarity:Contains 1 C1q domain.,similarity:Contains 1 collagen-like domain.,
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