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Mouse Anti-EHHADH Recombinant Antibody (CBFYE-0620) (V2LY-0425-LY1605)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYE-0620
Antibody Isotype
IgG1
Application
WB, IHC

Basic Information

Immunogen
Human recombinant protein fragment corresponding to amino acids 496-723 of human EHHADH produced in E.coli.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:500
IHC1:150

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
BSA & Glycerol & PBS
Preservative
Sodium Azide
Concentration
1 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase
Entrez Gene ID
1962
UniProt ID
Q08426
Research Area
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 (By similarity).

With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity (PubMed:15060085).

Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways (By similarity).

Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation (PubMed:15060085).
Biological Process
Fatty acid beta-oxidation Source: UniProtKB
Fatty acid beta-oxidation using acyl-CoA oxidase Source: Reactome
Cellular Location
Peroxisome
Involvement in disease
Fanconi renotubular syndrome 3 (FRTS3):
A form of Fanconi renotubular syndrome, a disease due to a generalized dysfunction of the proximal kidney tubule resulting in decreased solute and water reabsorption. Patients have polydipsia and polyuria with phosphaturia, glycosuria and aminoaciduria. They may develop hypophosphatemic rickets or osteomalacia, acidosis and a tendency toward dehydration. Some eventually develop renal insufficiency. FRTS3 inheritance is autosomal dominant.
PTM
Acetylated, leading to enhanced enzyme activity. Acetylation is enhanced by up to 80% after treatment either with trichostin A (TSA) or with nicotinamide (NAM) with highest increase on Lys-346. Acetylation and enzyme activity increased by about 1.5% on addition of fatty acids.
More Infomation

Forst, A. L., Reichold, M., Kleta, R., & Warth, R. (2021). Distinct Mitochondrial Pathologies Caused by Mutations of the Proximal Tubular Enzymes EHHADH and GATM. Frontiers in Physiology, 1119.

Ranea-Robles, P., Violante, S., Argmann, C., Dodatko, T., Bhattacharya, D., Chen, H., ... & Houten, S. M. (2021). Murine deficiency of peroxisomal L-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol homeostasis. Cellular and Molecular Life Sciences, 78(14), 5631-5646.

Okamura, S., Yoshino, H., Kuroshima, K., Tsuruda, M., Osako, Y., Sakaguchi, T., ... & Enokida, H. (2021). EHHADH contributes to cisplatin resistance through regulation by tumor-suppressive microRNAs in bladder cancer. BMC cancer, 21(1), 1-13.

Ranea-Robles, P., Portman, K., Bender, A., Lee, K., He, J. C., Mulholland, D. J., ... & Houten, S. M. (2021). Deficiency of peroxisomal L-bifunctional protein (EHHADH) causes male-specific kidney hypertrophy and proximal tubular injury in mice. bioRxiv.

Cui, J., Yi, G., Li, J., Li, Y., & Qian, D. (2021). Increased EHHADH Expression Predicting Poor Survival of Osteosarcoma by Integrating Weighted Gene Coexpression Network Analysis and Experimental Validation. BioMed Research International, 2021.

Luo, N., Chang, Q., Ren, X., Huang, P., Liu, W., Zhou, L., ... & Liu, J. (2020). Trichloroethylene injures rat liver and elevates the level of peroxisomal bifunctional enzyme (Ehhadh). Molecular & Cellular Toxicology, 16(3), 331-345.

Zhou, T., Xu, G., Sun, L., Yu, Z., & Wang, G. (2019). Improving Energy Metabolism of Deproteinized Extract of Calf Blood Through Regulation of Hmgcs2, Cpt1a, Angptl4, Cyp8b1, and Ehhadh Genes in Mice. Chemical Research in Chinese Universities, 35(3), 427-433.

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For research use only. Not intended for any clinical use.

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