Reactive species
Human;Rat
Applications
WB;IHC;IF;ELISA
Antibody type
Polyclonal Antibody
Protein name
Peroxisomal bifunctional enzyme
Immunogen
The antiserum was produced against synthesized peptide derived from human EHHADH. AA range:476-525
Specificity
PBFE Polyclonal Antibody detects endogenous levels of PBFE protein.
Constitute
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source
Polyclonal, Rabbit,IgG
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/10000.. IF 1:50-200
Purification process
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Other name
EHHADH; ECHD; Peroxisomal bifunctional enzyme; PBE; PBFE
Background
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.,
Function
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.,