Mouse HADHSC Monoclonal Antibody | anti-HADHSC antibody
HADHSC Conjugated Antibody
AF350 conjugated: most applications: 1:50-1:250
AF405 conjugated: most applications: 1:50-1:250
AF488 conjugated: most applications: 1:50-1:250
AF555 conjugated: most applications: 1:50-1:250
AF594 conjugated: most applications: 1:50-1:250
AF647 conjugated: most applications: 1:50-1:250
AF680 conjugated: most applications: 1:50-1:250
AF750 conjugated: most applications: 1:50-1:250
Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000
NCBI and Uniprot Product Information
NCBI Description
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 of this gene on chromosome 15. [provided by RefSeq, May 2010]
Uniprot Description
HADHSC: Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA. Defects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency). HADH deficiency is a metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death. Defects in HADH are the cause of familial hyperinsulinemic hypoglycemia type 4 (HHF4); also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or congenital hyperinsulinism. HHF is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. 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. Belongs to the 3-hydroxyacyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Amino Acid Metabolism - lysine degradation; Lipid Metabolism - fatty acid elongation in mitochondria; Carbohydrate Metabolism - butanoate; Oxidoreductase; Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Amino Acid Metabolism - tryptophan; EC 1.1.1.35
Chromosomal Location of Human Ortholog: 4q22-q26
Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; mitochondrial inner membrane; cytoplasm
Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity
Biological Process: response to drug; fatty acid beta-oxidation; response to activity; cellular lipid metabolic process; negative regulation of insulin secretion; response to insulin stimulus
Disease: 3-hydroxyacyl-coa Dehydrogenase Deficiency; Hyperinsulinemic Hypoglycemia, Familial, 4
Research Articles on HADHSC
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Product Notes
The HADHSC hadh (Catalog #AAA9456254) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The HADHSC Conjugated Antibody reacts with Human, Mouse, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's HADHSC can be used in a range of immunoassay formats including, but not limited to, Most Applications. Suggested Dilution: AF350 conjugated: most applications: 1:50-1:250 AF405 conjugated: most applications: 1:50-1:250 AF488 conjugated: most applications: 1:50-1:250 AF555 conjugated: most applications: 1:50-1:250 AF594 conjugated: most applications: 1:50-1:250 AF647 conjugated: most applications: 1:50-1:250 AF680 conjugated: most applications: 1:50-1:250 AF750 conjugated: most applications: 1:50-1:250 Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000. Researchers should empirically determine the suitability of the HADHSC hadh 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 "HADHSC, 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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