Mouse Anti-EHHADH (AA 496-723) Recombinant Antibody (CBFYE-0618) (CBMAB-E1021-FY)

Name: Mouse Anti-EHHADH (AA 496-723) Recombinant Antibody (CBFYE-0618)
SKU: CBMAB-E1021-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

CBFYE-0618

Application

WB, IHC

Immunogen

Human recombinant protein fragment corresponding to amino acids 496-723 of human EHHADH produced in E.coli

Specificity

Human

Antibody Isotype

IgG1

Clonality

Monoclonal

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

Buffer

PBS, pH 7.3, 1%BSA, 50% glycerol

Preservative

0.02% Sodium azide

Concentration

1 mg/mL

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Epitope

AA 496-723

More Infomation

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.

Entrez Gene ID

1962

UniProt ID

Q08426

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Alternative Versions

Mouse Anti-EHHADH Recombinant Antibody (CBFYE-0618) (CAT#: V2LY-0425-LY1603)

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Mouse Anti-EHHADH Recombinant Antibody (CBFYE-0618) (CAT#: V2LY-0425-LY1603)

Isotype control

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

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