Name: HADHA (F9M4C) Rabbit Monoclonal Antibody
Price: 161.00 USD
Availability: InStock

Filter:

WB

Product Specifications

REACTIVITY	H
SENSITIVITY	Endogenous
MW (kDa)	80
Source/Isotype	Rabbit IgG

Application Key:

WB-Western Blotting

Species Cross-Reactivity Key:

H-Human

Product Information

Product Usage Information

Application	Dilution
Western Blotting	1:1000
Simple Western™	1:50 - 1:250

Storage

Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.

实验步骤

Specificity / Sensitivity

HADHA (F9M4C) Rabbit Monoclonal Antibody recognizes endogenous levels of total HADHA protein. This antibody does not cross-react with HADHB protein.

Species Reactivity:

Human

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val168 of human HADHA protein.

Background

Mitochondrial trifunctional enzyme, also known as mitochondrial trifunctional protein (MTP), catalyzes three reactions in the fatty acid β-oxidation pathway. It has two different types of subunits: α/HADHA and β/HADHB. HADHA possesses the 2,3-enoyl-CoA hydratase (ECH) and 3-hydroxyacyl-CoA dehydrogenase (HACD) activities, while HADHB has 3-ketoacyl-CoA thiolase activity (1-3). Studies show that HADHA stimulates ketone body production through fatty acid β-oxidation. β-hydroxybutyrate, one of the ketone bodies, inhibits gluconeogenesis (1). Furthermore, studies have identified mutations in HADHB that cause MTP deficiency, an autosomal recessive disorder severely restricting long-chain fatty acid oxidation (4-6).

Alternate Names

3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit; 3-oxoacyl-CoA thiolase; 78 kDa gastrin-binding protein; ECHA; gastrin-binding protein; GBP; HADH; HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; LCEH; LCHAD; Long chain 3-hydroxyacyl-CoA dehydrogenase; long-chain 2-enoyl-CoA hydratase; Long-chain enoyl-CoA hydratase; long-chain-3-hydroxyacyl-CoA dehydrogenase; MGC1728; mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit; mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit; mitochondrial trifunctional enzyme, alpha subunit; mitochondrial trifunctional protein, alpha subunit; Monolysocardiolipin acyltransferase; MTPA; TP-alpha; Trifunctional enzyme subunit alpha, mitochondrial

Database Links

UniProt ID: P40939

Entrez-Gene Id: 3030

限制使用

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For Research Use Only. Not for Use in Diagnostic Procedures.

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HADHA (F9M4C) Rabbit Monoclonal Antibody #50257