Target：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

Gene Name：EHHADH

Protein Name：Peroxisomal bifunctional enzyme

Human Gene Id：1962

Human Swiss Prot No：Q08426

Mouse Swiss Prot No：Q9DBM2

Rat Gene Id：171142

Rat Swiss Prot No：P07896

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.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:10000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：EHHADH;ECHD;Peroxisomal bifunctional enzyme;PBE;PBFE

Observed Band(KD)：80kD

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.,

Subcellular Location：Peroxisome .

Expression：Liver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain.