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Mouse Anti-HADHSC Recombinant Antibody (3C9) (CBMAB-A3724-LY)

The product is antibody recognizes HADHSC. The antibody 3C9 immunoassay techniques such as: WB, ELISA.
See all HADHSC antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
3C9
Antibody Isotype
IgG2b, κ
Application
WB, ELISA

Basic Information

Immunogen
HADHSC (NP_005318, 205 a.a. ~ 314 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human
Antibody Isotype
IgG2b, κ
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
Purity
> 95% Purity determined 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
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. [provided by RefSeq]
Entrez Gene ID
3033
UniProt ID
Q16836
Alternative Names
HAD; HADH1; HADHSC; HHF4; M/SCHAD; MGC8392; SCHAD
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.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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