EHHADH Antibody

Code CSB-PA003717
Size US$100
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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
EHHADH
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.

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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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