Molecular weight (DA)
80kD
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.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/10000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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.,
Protein name
Peroxisomal bifunctional enzyme
Other name
EHHADH; ECHD; Peroxisomal bifunctional enzyme; PBE; PBFE
Fields
>>Fatty acid degradation;>>Valine, leucine and isoleucine degradation;>>Lysine degradation;>>Tryptophan metabolism;>>beta-Alanine metabolism;>>Propanoate metabolism;>>Butanoate metabolism;>>Metabolic pathways;>>Fatty acid metabolism;>>PPAR signaling pathway;>>Peroxisome
Human protein sequence Database
Q08426
Mouse protein sequence database
Q9DBM2
Rat protein sequence database
P07896
Cellular localization
Peroxisome .
Tissue expression
Liver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain.
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