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Rabbit Anti-EHHADH Recombinant Antibody (EG1056) (CBMAB-EN1246-LY)

The product is antibody recognizes EHHADH. The antibody EG1056 immunoassay techniques such as: WB: 1:500~1:1000 IHC: 1:50~1:100 ELISA: 1:40000.
See all EHHADH antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse
Clone
EG1056
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 IHC: 1:50~1:100 ELISA: 1:40000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human EHHADH.
Specificity
Human, Mouse
Antibody Isotype
IgG
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Format
Liquid
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
Introduction
The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
Entrez Gene ID
Human1962
Mouse74147
UniProt ID
HumanQ08426
MouseQ9DBM2
Alternative Names
Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase; Enoyl-Coenzyme A, Hydratase/3-Hydroxyacyl Coenzyme A Dehydrogenase; ECHD; PBFE; PBE; Enoyl-CoA, Hydratase/3-Hydroxyacyl CoA Dehydrogenase; L-Bifunctional Protein, Peroxisomal; L-3-Hydroxyacyl-CoA Dehydrogenase;
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.

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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