Hydroxyacyl-coenzyme A dehydrogenase Recombinant Protein | Hadh recombinant protein

Recombinant Mouse Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial

Cat. Num. AAA1303917

• Gene Names: Hadh; HCDH; Schad; Hadhsc; AA409008; AU019341; AW742602
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: Hydroxyacyl-coenzyme A dehydrogenase; Recombinant Mouse Hydroxyacyl-coenzyme A dehydrogenase; mitochondrial; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase; Hadh recombinant protein

• Host: E Coli or Yeast or Baculovirus or Mammalian Cell
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized or liquid (Format to be determined during the manufacturing process)
• Sequence Positions: 13-314aa; Full Length
• Sequence: SSSSSASAAAKKILIKHVTVIGGGLMGAGIAQVAAATGHTVVLVDQTEDILAKSKKGIEESLKRMAKKKFTENPKAGDEFVEKTLSCLSTSTDAASVVHSTDLVVEAIVENLKLKNELFQRLDKFAAEHTIFASNTSSLQITNIANATTRQDRFAGLHFFNPVPMMKLVEVIKTPMTSQKTFESLVDFCKTLGKHPVSCKDTPGFIVNRLLVPYLIEAVRLHERGDASKEDIDTAMKLGAGYPMGPFELLDYVGLDTTKFILDGWHEMEPENPLFQPSPSMNNLVAQKKLGKKTGEGFYKYK
• Sequence Length: 314

• Preparation and Storage: Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

SDS-PAGE

Related Product Information for Hadh recombinant protein

Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.

References

Isolation of a cDNA clone encoding mouse 3-hydroxyacyl CoA dehydrogenase.Nomura M., Takihara Y., Shimada K.Gene 160:309-310(1995) Mouse medium and short chain L-3-hydroxyacyl-coenzyme A dehydrogenase gene.O'Brien L.K., Sims H.F., Strauss A.W. SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways.Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.Mol. Cell 50:919-930(2013) Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways.Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013)

NCBI and Uniprot Product Information

• NCBI GI #: 111038118
• NCBI GeneID: 15107
• NCBI Accession #: NP_032238.2
• NCBI GenBank Nucleotide #: NM_008212.4
• UniProt Accession #: Q61425; Q3TF75; Q3THK8; Q3UFI0; Q8K149; Q925U9
• Molecular Weight: 49 kDa
• NCBI Official Full Name: hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
• NCBI Official Synonym Full Names: hydroxyacyl-Coenzyme A dehydrogenase
• NCBI Official Symbol: Hadh
• NCBI Official Synonym Symbols: HCDH; Schad; Hadhsc; AA409008; AU019341; AW742602
• 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: Hadhsc; Mschad; Schad; HCDH
• UniProt Entry Name: HCDH_MOUSE

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

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

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity; oxidoreductase activity

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