Mouse Anti-HADHB Recombinant Antibody (5C3AB7) (CBMAB-H1546-FY)

Name: Mouse Anti-HADHB Recombinant Antibody (5C3AB7)
SKU: CBMAB-H1546-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

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

Basic Information

Host Animal

Mouse

Clone

5C3AB7

Application

WB, IP, ICC, FC

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

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.

More Infomation

Target

Full Name

hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit

Introduction

This gene encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described.

Entrez Gene ID

3032

UniProt ID

P55084

Alternative Names

Hydroxyacyl-CoA Dehydrogenase Trifunctional Multienzyme Complex Subunit Beta; Hydroxyacyl-Coenzyme A Dehydrogenase/3-Ketoacyl-Coenzyme A Thiolase/Enoyl-Coenzyme A Hydratase (Trifunctional Protein), Beta Subunit; Hydroxyacyl-CoA Dehydrogenase/3-Ketoacyl-CoA Thiolase/Enoyl-CoA Hydratase (Trifunctional Protein), Beta Subunit; TP-BETA; 3-Ketoacyl-Coenzyme A (CoA) Thiolase Of Mitochondrial Trifunctional Protein, Beta Subunit; Mitochondrial Trifunctional Protein, Beta Subunit; 2-Enoyl-Coenzyme A (CoA) Hydratase, Beta Subunit; Trifunctional Enzyme Subunit Beta, Mitochondrial

Function

Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:8135828, PubMed:29915090, PubMed:30850536).

The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA (PubMed:29915090).

Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed:30850536).

Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3-ketoacyl-CoA thiolase activity (PubMed:8135828, PubMed:29915090, PubMed:30850536).

Biological Process

Fatty acid beta-oxidation Source: ComplexPortal

Cellular Location

Mitochondrion; Mitochondrion inner membrane; Mitochondrion outer membrane; Endoplasmic reticulum. Protein stability and association with membranes require HADHA.

Involvement in disease

Mitochondrial trifunctional protein deficiency (MTPD):
A disease biochemically characterized by loss of all enzyme activities of the mitochondrial trifunctional protein complex. Variable clinical manifestations include hypoglycemia, cardiomyopathy, delayed psychomotor development, sensorimotor axonopathy, generalized weakness, hepatic dysfunction, respiratory failure. Sudden infant death may occur. Most patients die from heart failure.

Sekine, Y., Yamamoto, K., Kurata, M., Honda, A., Onishi, I., Kinowaki, Y., ... & Kitagawa, M. (2022). HADHB, a fatty acid beta-oxidation enzyme, is a potential prognostic predictor in malignant lymphoma. Pathology, 54(3), 286-293.

Ørstavik, K., Arntzen, K. A., Mathisen, P., Backe, P. H., Tangeraas, T., Rasmussen, M., ... & Bliksrud, Y. T. (2022). Novel mutations in the HADHB gene causing a mild phenotype of mitochondrial trifunctional protein (MTP) deficiency. JIMD reports, 63(3), 193-198.

Dagher, R., Massie, R., & Gentil, B. J. (2021). MTP deficiency caused by HADHB mutations: Pathophysiology and clinical manifestations. Molecular Genetics and Metabolism, 133(1), 1-7.

Shao, F., Wang, X., Yu, J., Shen, K., Qi, C., & Gu, Z. (2019). Expression of miR-33 from an SREBP2 intron inhibits the expression of the fatty acid oxidation-regulatory genes CROT and HADHB in chicken liver. British poultry science, 60(2), 115-124.

Liu, Z. R., Dong, H. L., Ma, Y., & Wu, Z. Y. (2019). Identification and functional characterization of mutations within HADHB associated with mitochondrial trifunctional protein deficiency. Mitochondrion, 49, 200-205.

Li, J., Suda, K., Ueoka, I., Tanaka, R., Yoshida, H., Okada, Y., ... & Yamaguchi, M. (2019). Neuron-specific knockdown of Drosophila HADHB induces a shortened lifespan, deficient locomotive ability, abnormal motor neuron terminal morphology and learning disability. Experimental Cell Research, 379(2), 150-158.

Velasco, A. D. R., Welten, S. M., Goossens, E. A., Quax, P. H., Rappsilber, J., Michlewski, G., & Nossent, A. Y. (2019). Posttranscriptional Regulation of 14q32 MicroRNAs by the CIRBP and HADHB during Vascular Regeneration after Ischemia. Molecular Therapy-Nucleic Acids, 14, 329-338.

Diebold, I., Schön, U., Horvath, R., Schwartz, O., Holinski-Feder, E., Kölbel, H., & Abicht, A. (2019). HADHA and HADHB gene associated phenotypes-Identification of rare variants in a patient cohort by Next Generation Sequencing. Molecular and cellular probes, 44, 14-20.

Zhu, Y., Lu, H., Zhang, D., Li, M., Sun, X., Wan, L., ... & Lai, M. (2018). Integrated analyses of multi-omics reveal global patterns of methylation and hydroxymethylation and screen the tumor suppressive roles of HADHB in colorectal cancer. Clinical epigenetics, 10(1), 1-13.

Lu, Y., Wu, R., Meng, L., He, L., Liu, J., Zuo, Y., ... & Wang, Z. (2018). HADHB mutations cause infantile-onset axonal Charcot-Marie-Tooth disease: A report of two cases. Clinical Neuropathology, 37(5), 232.

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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)
Proteogenomic
Other Protocols

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

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