Mouse Anti-HADH Recombinant Antibody (CBFYH-2849) (CBMAB-H1536-FY)

Name: Mouse Anti-HADH Recombinant Antibody (CBFYH-2849)
SKU: CBMAB-H1536-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

CBFYH-2849

Application

IHC-P, WB

Specificity

Human

Antibody Isotype

IgG2a

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

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-CoA Dehydrogenase

Introduction

This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene of this gene on chromosome 15.

Entrez Gene ID

3033

UniProt ID

Q16836

Alternative Names

Hydroxyacyl-CoA Dehydrogenase; Medium And Short-Chain L-3-Hydroxyacyl-Coenzyme A Dehydrogenase; L-3-Hydroxyacyl-Coenzyme A Dehydrogenase, Short Chain; Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase; EC 1.1.1.35; HADHSC; SCHAD; HCDH; HAD

Function

Mitochondrial fatty acid beta-oxidation enzyme that catalyzes the third step of the beta-oxidation cycle for medium and short-chain 3-hydroxy fatty acyl-CoAs (C4 to C10) (PubMed:10231530, PubMed:11489939, PubMed:16725361).

Plays a role in the control of insulin secretion by inhibiting the activation of glutamate dehydrogenase 1 (GLUD1), an enzyme that has an important role in regulating amino acid-induced insulin secretion (By similarity).

Biological Process

Fatty acid beta-oxidation Source: UniProtKB
Negative regulation of insulin secretion Source: Ensembl
Positive regulation of cold-induced thermogenesis Source: YuBioLab
Regulation of insulin secretion Source: UniProtKB
Response to activity Source: Ensembl
Response to insulin Source: Ensembl
Response to xenobiotic stimulus Source: Ensembl

Cellular Location

Mitochondrion matrix

Involvement in disease

3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency):
An autosomal recessive, metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death.
Familial hyperinsulinemic hypoglycemia 4 (HHF4):
Most common cause of persistent hypoglycemia in infancy. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. HHF4 should be easily recognizable by analysis of acylcarnitine species and that this disorder responds well to treatment with diazoxide. It provides the first 'experiment of nature' that links impaired fatty acid oxidation to hyperinsulinism and that provides support for the concept that a lipid signaling pathway is implicated in the control of insulin secretion.

PTM

Succinylation at Lys-81, adjacent to a coenzyme A binding site. Desuccinylated by SIRT5.

Fang, H., Li, H., Zhang, H., Wang, S., Xu, S., Chang, L., ... & Cui, R. (2022). Short-chain L-3-hydroxyacyl-CoA dehydrogenase: A novel vital oncogene or tumor suppressor gene in cancers. Frontiers in Pharmacology, 13, 1019312.

Xia, G., Gao, Y., Wu, C., Pan, H., Hou, J., Su, J., ... & Gao, X. (2022). A novel isoform of hydroxyacyl-CoA dehydrogenase inhibits cell proliferation. Biochemical and Biophysical Research Communications, 606, 75-79.

Karunadasa, A. K. U. I., Toma, C., Senaratne, K. M. P. H., Kumara, K. G. R. A., & Gamage, C. D. (2021). Leptospiral 3-hydroxyacyl-CoA dehydrogenase as an early urinary biomarker of leptospirosis in a Sri Lankan setting–Interim results. Sri Lankan Journal of Infectious Diseases, 11.

Wongkittichote, P., Watson, J. R., Leonard, J. M., Toolan, E. R., Dickson, P. I., & Grange, D. K. (2020). Fatal COVID‐19 infection in a patient with long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency: a case report. JIMD reports, 56(1), 40-45.

Bernardi, C., Tirloni, E., Stella, S., Anastasio, A., Cattaneo, P., & Colombo, F. (2019). ß-hydroxyacyl-CoA-dehydrogenase activity differentiates unfrozen from frozen-thawed Yellowfin tuna (Thunnus albacares). Italian journal of food safety, 8(3).

Fraser, H., Geppert, J., Johnson, R., Johnson, S., Connock, M., Clarke, A., ... & Stinton, C. (2019). Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficiency: a systematic review. Orphanet Journal of Rare Diseases, 14(1), 1-18.

Segawa, M., Wen, C., Orita, I., Nakamura, S., & Fukui, T. (2019). Two NADH-dependent (S)-3-hydroxyacyl-CoA dehydrogenases from polyhydroxyalkanoate-producing Ralstonia eutropha. Journal of bioscience and bioengineering, 127(3), 294-300.

Toma, C., Koizumi, N., Kakita, T., Yamaguchi, T., Hermawan, I., Higa, N., & Yamashiro, T. (2018). Leptospiral 3-hydroxyacyl-CoA dehydrogenase as an early urinary biomarker of leptospirosis. Heliyon, 4(4).

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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

Antibody Pairs

HADH Matched Antibody Pair (523) (CAT#: APMAB-523LY)

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Isotype control

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

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