Western Blot (WB)
(Western Blot analysis of HEK293 cells using HADHA Monoclonal Antibody at dilution of 1:4000 )
Mouse anti-Human, Mouse HADHA Monoclonal Antibody | anti-HADHA antibody
HADHA Monoclonal Antibody
Immunohistochemistry:1:20-1:200
Western Blot (WB)
(Western Blot analysis of HEK293 cells using HADHA Monoclonal Antibody at dilution of 1:4000 )
Western Blot (WB)
(Western Blot analysis of HEK293 cells using HADHA Monoclonal Antibody at dilution of 1:4000 )
NCBI and Uniprot Product Information
NCBI Description
This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008]
Uniprot Description
HADHA: Bifunctional subunit. Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency). The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all enzyme activities of the TFP complex. Defects in HADHA are the cause of long-chain 3-hydroxyl- CoA dehydrogenase deficiency (LCHAD deficiency). The clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long- chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced. Defects in HADHA are a cause of maternal acute fatty liver of pregnancy (AFLP). AFLP is a severe maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome).
Protein type: Lipid Metabolism - unsaturated fatty acid biosynthesis; Acetyltransferase; EC 4.2.1.17; Amino Acid Metabolism - lysine degradation; Carbohydrate Metabolism - propanoate; Secondary Metabolites Metabolism - limonene and pinene degradation; Other Amino Acids Metabolism - beta-alanine; Carbohydrate Metabolism - butanoate; Lipid Metabolism - fatty acid elongation in mitochondria; Lyase; Lipid Metabolism - fatty acid; EC 1.1.1.211; Oxidoreductase; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial
Chromosomal Location of Human Ortholog: 2p23
Cellular Component: mitochondrial inner membrane; mitochondrion
Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity; acetyl-CoA C-acetyltransferase activity; protein binding
Biological Process: fatty acid beta-oxidation
Disease: Long-chain 3-hydroxyacyl-coa Dehydrogenase Deficiency; Trifunctional Protein Deficiency
Research Articles on HADHA
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Product Notes
The HADHA hadha (Catalog #AAA2540199) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The HADHA Monoclonal Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's HADHA can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC). Western Blot: 1:500-1:5000 Immunohistochemistry:1:20-1:200. Researchers should empirically determine the suitability of the HADHA hadha for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "HADHA, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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