PBFE rabbit pAb
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.
Product Specifications
Background
UniProt
Q08426
Swiss Prot
Q08426
Reactivity
Human; Rat
Immunogen
The antiserum was produced against synthesized peptide derived from human EHHADH. AA range:476-525
Clonality
Polyclonal
Source
Rabbit
Applications
WB; IHC; IF; ELISA
Concentration
1 mg/ml
Dilution
Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. ELISA: 1/10000. Not yet tested in other applications.
Molecular Weight
80kD
Storage Conditions
-20°C/1 year
Observed Molecular Weight
80kD
Fragment
IgG
Subcellular Location
Peroxisome .
Other Product Names
EHHADH; ECHD; Peroxisomal bifunctional enzyme; PBE; PBFE
Gene ID (Human)
1962
Available Sizes
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