Rabbit HADH Polyclonal Antibody | anti-HADH antibody

HADH Rabbit Polyclonal

Cat. Num. AAA769979

• Gene Names: HADH; HAD; HCDH; HHF4; HADH1; SCHAD; HADHSC; MSCHAD
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry, Immunoprecipitation
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: HADH; Polyclonal Antibody; HADH Rabbit Polyclonal; anti-HADH antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 314

• Applicable Applications for anti-HADH antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunoprecipitation (IP)
• Immunogen: Hydroxyacyl-Coenzyme A dehydrogenase
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human liver cancer using MBS769979 (HADH antibody) at dilution of 1:100)

Western Blot (WB)

(HepG2 cells were subjected to SDS PAGE followed by western blot with MBS769979 (HADH antibody) at dilution of 1:1000)

NCBI and Uniprot Product Information

• NCBI GI #: 296179429
• NCBI GeneID: 3033
• NCBI Accession #: NP_005318.3
• NCBI GenBank Nucleotide #: NM_005327.4
• Molecular Weight: 36,051 Da
• NCBI Official Full Name: hydroxyacyl-coenzyme A dehydrogenase, mitochondrial isoform 2
• NCBI Official Synonym Full Names: hydroxyacyl-CoA dehydrogenase
• NCBI Official Symbol: HADH
• NCBI Official Synonym Symbols: HAD; HCDH; HHF4; HADH1; SCHAD; HADHSC; MSCHAD
• NCBI Protein Information: hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
• UniProt Protein Name: Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
• Protein Family: Hydroxyacyl-coenzyme A dehydrogenase
• UniProt Gene Name: HADH
• UniProt Synonym Gene Names: HAD; HADHSC; SCHAD; HCDH
• UniProt Entry Name: HCDH_HUMAN

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: Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.1.1.35; Lipid Metabolism - fatty acid elongation in mitochondria; Oxidoreductase; Lipid Metabolism - fatty acid

Chromosomal Location of Human Ortholog: 4q22-q26

Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrial matrix; mitochondrion; nucleoplasm

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity

Biological Process: fatty acid beta-oxidation; negative regulation of insulin secretion; response to activity; response to drug; response to insulin stimulus

Disease: 3-hydroxyacyl-coa Dehydrogenase Deficiency; Hyperinsulinemic Hypoglycemia, Familial, 4