蛋白名称
Peroxisomal bifunctional enzyme
免疫原
The antiserum was produced against synthesized peptide derived from human EHHADH. AA range:476-525
特异性
PBFE Monoclonal Antibody detects endogenous levels of PBFE protein.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
其他名称
EHHADH; ECHD; Peroxisomal bifunctional enzyme; PBE; PBFE
背景
catalytic activity:(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H(2)O.,catalytic activity:(3Z)-dodec-3-enoyl-CoA = (2E)-dodec-2-enoyl-CoA.,catalytic activity:(S)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.,disease:Absent in patients suffering with peroxisomal disorders such as Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease.,pathway:Lipid metabolism; fatty acid beta-oxidation.,similarity:In the C-terminal section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.,similarity:In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.,subunit:Monomer.,tissue specificity:Liver and kidney. Lower amounts seen in the brain.,
功能
catalytic activity:(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H(2)O.,catalytic activity:(3Z)-dodec-3-enoyl-CoA = (2E)-dodec-2-enoyl-CoA.,catalytic activity:(S)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.,disease:Absent in patients suffering with peroxisomal disorders such as Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease.,pathway:Lipid metabolism; fatty acid beta-oxidation.,similarity:In the C-terminal section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.,similarity:In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.,subunit:Monomer.,tissue specificity:Liver and kidney. Lower amounts seen in the brain.,
