EHHADH Antibody

Code CSB-PA003717
Size US$119
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  • Western Blot analysis of A549 cells using PBFE Polyclonal Antibody
  • Western Blot analysis of Jurkat K562 NIH-3T3 cells using PBFE Polyclonal Antibody
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Product Details

Uniprot No.
Target Names
Alternative Names
3 hydroxyacyl CoA dehydrogenase antibody; 3,2 trans enoyl CoA isomerase antibody; 3-hydroxyacyl-CoA dehydrogenase antibody; ECHD antibody; ECHP_HUMAN antibody; EHHADH antibody; Enoyl Coenzyme A antibody; Enoyl Coenzyme A; hydratase/3 hydroxyacyl Coenzyme A dehydrogenase antibody; L 3 hydroxyacyl CoA dehydrogenase antibody; L bifunctional protein antibody; L bifunctional protein; peroxisomal antibody; L PBE antibody; LBFP antibody; LBP antibody; MGC120586 antibody; MS730 antibody; PBE antibody; PBFE antibody; Peroxisomal bifunctional enzyme antibody; Peroxisomal enoyl CoA hydratase antibody
Raised in
Rabbit
Species Reactivity
Human,Rat
Immunogen
Synthesized peptide derived from the Internal region of Human PBFE.
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Form
Liquid
Tested Applications
WB, IHC, ELISA
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:100-1:300
ELISA 1:10000
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
Peroxisomal trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. Catalyzes two of the four reactions of the long straight chain fatty acids peroxisomal beta-oxidation pathway. Optimal isomerase for 2,5 double bonds into 3,5 form isomerization in a range of enoyl-CoA species (Probable). Also able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species. With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity. Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways. Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation.
Gene References into Functions
  1. Mistargeting of peroxisomal EHHADH disrupts mitochondrial metabolism and leads to renal Fanconi's syndrome; this indicates a central role of mitochondria in proximal tubular function. PMID: 24401050
Involvement in disease
Fanconi renotubular syndrome 3 (FRTS3)
Subcellular Location
Peroxisome.
Protein Families
Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family
Tissue Specificity
Liver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain.
Database Links

HGNC: 3247

OMIM: 607037

KEGG: hsa:1962

STRING: 9606.ENSP00000231887

UniGene: Hs.429879

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7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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