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>   首页   >   KD-Validated Anti-HADHA Mouse Monoclonal Antibody   

KD-Validated Anti-HADHA Mouse Monoclonal Antibody

Mouse monoclonal antibody

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, FC
Primary Accession P40939
Reactivity Rat, Human, Mouse
Clonality Monoclonal
Isotype Mouse IgG1
Clone Names 24GB6655
Calculated MW 83000 Da
Gene Name HADHA
Aliases HADHA; Hydroxyacyl-CoA Dehydrogenase Trifunctional Multienzyme Complex Subunit Alpha; LCHAD; LCEH; MTPA; GBP; Hydroxyacyl-Coenzyme A Dehydrogenase/3-Ketoacyl-Coenzyme A Thiolase/Enoyl-Coenzyme A HydRatase (Trifunctional Protein), Alpha Subunit; Hydroxyacyl-CoA Dehydrogenase/3-Ketoacyl-CoA Thiolase/Enoyl-CoA HydRatase (Trifunctional Protein), Alpha Subunit; Mitochondrial Trifunctional Protein, Alpha Subunit; Trifunctional Enzyme Subunit Alpha, Mitochondrial; Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; Monolysocardiolipin Acyltransferase; Long-Chain 2-Enoyl-CoA HydRatase; 78 KDa Gastrin-Binding Protein; Gastrin-Binding Protein; HADH; Mitochondrial Long-Chain L-3-Hydroxyacyl-Coenzyme A (CoA) Dehydrogenase, Alpha Subunit; Mitochondrial Long-Chain 2-Enoyl-Coenzyme A (CoA) HydRatase, Alpha Subunit; 3-Ketoacyl-Coenzyme A (CoA) Thiolase, Alpha Subunit; Mitochondrial Trifunctional Enzyme, Alpha Subunit; 3-Oxoacyl-CoA Thiolase; EC 2.3.1.- ; TP-ALPHA; TP-Alpha; ECHA
Immunogen Recombinant protein of human HADHA
Additional Information
Gene ID 3030
Other Names Trifunctional enzyme subunit alpha, mitochondrial, 78 kDa gastrin-binding protein, Monolysocardiolipin acyltransferase, MLCL AT, 2.3.1.-, TP-alpha, Long-chain enoyl-CoA hydratase, 4.2.1.17, Long chain 3-hydroxyacyl-CoA dehydrogenase, 1.1.1.211, HADHA, HADH

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name HADHA
Synonyms HADH
Function Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:1550553, PubMed:29915090, PubMed:30850536, PubMed:8135828, PubMed:31604922). The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through cycles of four consecutive reactions (PubMed:29915090). Each beta-oxidation cycle shortens the fatty acyl-CoA by two carbons, yielding one acetyl-CoA (for the citric acid cycle), one FADH(2), and one NADH (which donate electrons to the respiratory chain for ATP production) (PubMed:29915090). These cycles repeat until the chain is fully degraded to acetyl-CoA units (PubMed:29915090). Among the enzymes involved in this pathway, the trifunctional protein--responsible for the hydration, dehydrogenation, and thiolysis steps, shows specificity for long-chain fatty acids, such as those from dietary and stored fats (PubMed:30850536, PubMed:31604922). Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity (Probable) (PubMed:29915090, PubMed:30850536, PubMed:8135828). These activities have been experimentally confirmed on a few substrates derived from beta- oxidation of long-chain saturated fatty acids such as palmitate (hexadecanoate) and laurate (dodecanoate) (PubMed:1550553, PubMed:8135828, PubMed:8163672, PubMed:8651282). In addition, based on its established catalytic mechanism, and combined genetic interaction or mutant phenotype evidence, it is predicted to act also on other substrates, including long-chain unsaturated fatty acids such as oleate (9Z-octadecenoate), linoleate (9Z,12Z-octadecadienoate), linolenate (9Z,12Z,15Z-octadecatrienoate), and others (Probable) (PubMed:26474213). Independently of subunit beta, HADHA also exhibits a cardiolipin acyltransferase activity that participates in cardiolipin remodeling; cardiolipin is a major mitochondrial membrane phospholipid (PubMed:23152787, PubMed:31604922). HADHA may act downstream of Tafazzin/TAZ, that remodels monolysocardiolipin (MLCL) to a cardiolipin intermediate, and then HADHA may continue to remodel this species into mature tetralinoleoyl-cardiolipin (PubMed:31604922). Has also been proposed to act directly on MLCL; capable of acylating MLCL using different acyl-CoA substrates, with highest activity for oleoyl-CoA (PubMed:23152787).
Cellular Location Mitochondrion. Mitochondrion inner membrane Note=Protein stability and association with mitochondrion inner membrane do not require HADHB.
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