protein

HADH, 13-314aa, Human, His tag, E Coli

MBS203557

0.1 mg

285 USD

Recombinant Protein

HADH, 13-314aa, Human, His tag, E Coli

HADH

HAD; HADH1; HHF4; M/SCHAD; SCHAD; Hydroxyacyl-coenzyme A dehydrogenase; mitochondrial Hydroxyacyl Coenzyme A dehydrogenase type II; Mitochondrial L3 Hydroxyacyl CoA Dehydrogenase; 17 beta hydroxysteroid dehydrogenase 10; 17 beta hydroxysteroid dehydrogenase type 10; 3 hydroxy 2 methylbutyryl CoA dehydrogenase; 3 hydroxyacyl CoA dehydrogenase type 2; 3 hydroxyacyl CoA dehydrogenase type II; AB binding alcohol dehydrogenase; 17b HSD10; ABAD; Ads9; Amyloid beta binding polypeptide; Amyloid beta peptide binding alcohol dehydrogenase; Amyloid beta peptide binding protein; CAMR; DuPXp11.22; Endoplasmic Reticulum Amyloid Binding Protein; Endoplasmic reticulum associated amyloid beta peptide binding protein; ER associated a+myloid beta-binding protein; ERAB; HADH 2; HADH2; HCD 2; HCD2; HSD17B10; Hydroxyacyl CoA Dehydrogenase type II; Hydroxysteroid (17 beta) dehydrogenase 10; Mental retardation X linked syndromic 11; MHBD; Mitochondrial ribonuclease P protein 2; Mitochondrial RNase P protein 2; MRPP2; MRX17; Short chain dehydrogenase/reductase family 5C member 1; SDR5C1; Short chain L 3 hydroxyacyl CoA dehydrogenase type 2; Short chain type dehydrogenase/reductase XH98G2; Type 10 17b HSD; Type 10 17beta hydroxysteroid dehydrogenase; Type II HADH; XH98G2.

Hydroxyacyl-Coenzyme A dehydrogenase; Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain; medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; short-chain 3-hydroxyacyl-CoA dehydrogenase; hydroxyacyl-CoA dehydrogenase; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase

HADH

HADH; HADH; HAD; HCDH; HHF4; HADH1; SCHAD; HADHSC; MSCHAD; HAD; HADHSC; SCHAD; HCDH

HCDH_HUMAN

314

MGSSHHHHHH SSGLVPRGSH MSSSSTASAS AKKIIVKHVT VIGGGLMGAG IAQVAAATGH TVVLVDQTED ILAKSKKGIE ESLRKVAKKK FAENPKAGDE FVEKTLSTIA TSTDAASVVH STDLVVEAIV ENLKVKNELF KRLDKFAAEH TIFASNTSSL QITSIANATT RQDRFAGLHF FNPVPVMKLV EVIKTPMTSQ KTFESLVDFS KALGKHPVSC KDTPGFIVNR LLVPYLMEAI RLYERGDASK EDIDTAMKLG AGYPMGPFEL LDYVGLDTTK FIVDGWHEMD AENPLHQPSP SLNKLVAENK FGKKTGEGFY KYK

> 95% by SDS - PAGE

Liquid. 20mM Tris-HCl buffer (pH8.0) containing 20% glycerol, 0.1M NaCl

1.0 mg/ml (determined by Bradford assay)

Can be stored at 4 degree C short term (1-2 weeks). For long term storage, aliquot and store at -20 degree C or -70 degree C. Avoid repeated freezing and thawing cycles.

SDS-PAGE

Antigen Species: Human. Tag: His-tag

Expression System: E Coli

Metabolism

HADH, which belongs to the family of oxidoreductases, is important for converting certain fats to energy. This protein is an enzyme that catalyzes the chemical reaction. ((S)-3-hydroxyacyl-CoA + NAD+ 3-oxoacyl-CoA + NADH + H+) It is also involved in a process called fatty acid oxidation, in which several enzymes work in a step-wise fashion to break down (metabolize) fats and convert them to energy. Recombinant HADH protein was expressed in E.coli and purified by using conventional chromatography techniques.

Tieu K, et al. (2004) Ann Neurol. 56(1):51-60. Vredendaal PJ, et al. (1996) Biochem Biophys Res Commun. 223(3):718-23.

12653081

AAH00306

35.1 kDa (323aa), confirmed by MALDI-TOF.

Beta Oxidation Of Butanoyl-CoA To Acetyl-CoA Pathway (106130); Beta Oxidation Of Decanoyl-CoA To Octanoyl-CoA-CoA Pathway (106127); Beta Oxidation Of Hexanoyl-CoA To Butanoyl-CoA Pathway (106129); Beta Oxidation Of Lauroyl-CoA To Decanoyl-CoA-CoA Pathway (106126); Beta Oxidation Of Octanoyl-CoA To Hexanoyl-CoA Pathway (106128); Butanoate Metabolism Pathway (83007); Butanoate Metabolism Pathway (391); FOXA2 And FOXA3 Transcription Factor Networks Pathway (137911); Fatty Acid Beta Oxidation Pathway (198865); Fatty Acid Biosynthesis Pathway (198873)

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]

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: Oxidoreductase; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - lysine degradation; EC 1.1.1.35; Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid elongation in mitochondria; Amino Acid Metabolism - tryptophan. Chromosomal Location of Human Ortholog: 4q22-q26. Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; cytoplasm; mitochondrial inner membrane. 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

0.1 mg

285 USD

0.5 mg

640