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Description
HADHA Recombinant Rabbit mAb (S-2345-8)Product Specification Host Rabbit Antigen HADHA Synonyms Trifunctional enzyme subunit alpha, mitochondrial; 78 kDa gastrin binding protein; Monolysocardiolipin acyltransferase (MLCL AT); TP alpha; HADH Immunogen Synthetic Peptide Location Mitochondrion Accession P40939 Clone Number S 2345 8 Antibody Type Recombinant mAb Isotype IgG Application WB, IHC P, ICC Reactivity Hu, Ms Positive Sample HeLa, Jurkat, HepG2, 293T, NIH 3T3, mouse heart, mouse
Product Specification
| Host | Rabbit |
| Antigen | HADHA |
| Synonyms | Trifunctional enzyme subunit alpha, mitochondrial; 78 kDa gastrin-binding protein; Monolysocardiolipin acyltransferase (MLCL AT); TP-alpha; HADH |
| Immunogen | Synthetic Peptide |
| Location | Mitochondrion |
| Accession | P40939 |
| Clone Number | S-2345-8 |
| Antibody Type | Recombinant mAb |
| Isotype | IgG |
| Application | WB, IHC-P, ICC |
| Reactivity | Hu, Ms |
| Positive Sample | HeLa, Jurkat, HepG2, 293T, NIH/3T3, mouse heart, mouse kidney, PC-12, rat heart, rat kidney |
| Predicted Reactivity | Pg |
| Purification | Protein A |
| Concentration | 0.5 mg/ml |
| Conjugation | Unconjugated |
| Physical Appearance | Liquid |
| Storage Buffer | PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300 |
| Stability & Storage | 12 months from date of receipt / reconstitution, -20 °C as supplied |
Dilution
| application | dilution | species |
| WB | 1:1000-1:5000 | Hu, Ms, Rt |
| IHC-P | 1:200 | Hu, Ms |
| ICC | 1:500 | Hu, Ms |
Background
HADHA, which stands for hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex alpha subunit, is a crucial protein involved in the mitochondrial beta-oxidation of fatty acids. It is a part of the trifunctional protein complex that catalyzes three sequential steps in the breakdown of long-chain fatty acids into acetyl-CoA, a key molecule in cellular energy production. This protein is essential for the proper metabolism of fats and is particularly important in tissues with high energy demands such as the liver and heart. Mutations in the HADHA gene can lead to severe metabolic disorders, including long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, which can cause symptoms ranging from hypoglycemia and cardiomyopathy to more severe neurological complications, highlighting its critical role in maintaining metabolic homeostasis.
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