catalog number :
MBS143938
products type :
Recombinant Protein
products full name :
Recombinant Human Acyl-Coenzyme A Dehydrogenase, C-4 to C-12
products short name :
Acyl-Coenzyme A Dehydrogenase
products name syn :
ACADM Human; Acyl-Coenzyme A Dehydrogenase, C-4 to C-12 Human Recombinant; ACADM; ACAD1; CAD; MCADH; MCAD; EC=1.3.99.3; Medium-chain specific acyl-CoA dehydrogenase; mitochondrial; FLJ18227; FLJ93013; FLJ99884
other names :
medium-chain specific acyl-CoA dehydrogenase, mitochondrial isoform a; Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; medium-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain; acyl-CoA dehydrogenase, C-4 to C-12 straight chain
products gene name :
ACADM
other gene names :
ACADM; ACADM; MCAD; ACAD1; MCADH; MCAD
uniprot entry name :
ACADM_HUMAN
sequence :
MGSSHHHHHH SSGLVPRGSH MKANRQREPG LGFSFEFTEQ QKEFQATARK FAREEIIPVA AEYDKTGEYP VPLIRRAWEL GLMNTHIPEN CGGLGLGTFD ACLISEELAY GCTGVQTAIE GNSLGQMPII IAGNDQQKKK YLGRMTEEPL MCAYCVTEPG AGSDVAGIKT KAEKKGDEYI INGQKMWITN GGKANWYFLL ARSDPDPKAP ANKAFTGFIV EADTPGIQIG RKELNMGQRC SDTRGIVFED VKVPKENVLI GDGAGFKVAM GAFDKTRPVV AAGAVGLAQR ALDEATKYAL ERKTFGKLLV EHQAISFMLA EMAMKVELAR MSYQRAAWEV DSGRRNTYYA SIAKAFAGDI ANQLATDAVQ ILGGNGFNTE YPVEKLMRDA KIYQIYEGTS QIQRLIVARE HIDKYKN.
purity :
Greater than 90.0% as determined by SDS-PAGE.
form :
The ACADM (0.5mg/ml) protein solution containing 20mM Tris-HCl pH-7.5, 0.1M NaCl & 20% glycerol. Sterile filtered clear solution.
storage stability :
ACADM Human although stable at 4 degree C for 1 week, should be stored desiccated below -18 degree C. Please prevent freeze thaw cycles.
products categories :
ENZYMES; Enzymes; Dehydrogenase
products description :
Introduction: ACADM enzyme is essential for the degradation a certain group of fats called medium-chain fatty acids. ACADM is essential for converting specific fatty acids to energy, mainly during fasting periods. ACADM functions in mitochondria, the energy-producing centers within cells. ACADM is localized in the mitochondria of numerous tissue types, predominantly the liver. Description: ACADM Recombinant Human produced in E Coli is a single, non-glycosylated peptide chain contining 417 amino acids (26-421 a.a) and having molecular mass of 45.9 kDa. The ACADM is fused to a 21 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.
ncbi acc num :
NP_000007.1
ncbi gb acc num :
NM_000016.5
ncbi mol weight :
47,020 Da
ncbi pathways :
Beta Oxidation Of Decanoyl-CoA To Octanoyl-CoA-CoA Pathway (106127); Beta Oxidation Of Octanoyl-CoA To Hexanoyl-CoA Pathway (106128); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); FOXA2 And FOXA3 Transcription Factor Networks Pathway (137911); Fatty Acid Beta Oxidation Pathway (198865); Fatty Acid Degradation Pathway (82935); Fatty Acid Degradation Pathway (296); Fatty Acid Metabolism Pathway (868084); Fatty Acid Metabolism Pathway (878045)
ncbi summary :
This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
uniprot summary :
ACADM: This enzyme is specific for acyl chain lengths of 4 to 16. Defects in ACADM are the cause of acyl-CoA dehydrogenase medium-chain deficiency (ACADMD). It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Other Amino Acids Metabolism - beta-alanine; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; Lipid Metabolism - fatty acid; Oxidoreductase; EC 1.3.8.7; Mitochondrial. Chromosomal Location of Human Ortholog: 1p31. Cellular Component: mitochondrion; mitochondrial matrix; axon; nucleus. Molecular Function: acyl-CoA dehydrogenase activity; identical protein binding; FAD binding. Biological Process: carnitine metabolic process, CoA-linked; fatty acid beta-oxidation; medium-chain fatty acid catabolic process; cellular lipid metabolic process; medium-chain fatty acid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; carnitine biosynthetic process. Disease: Acyl-coa Dehydrogenase, Medium-chain, Deficiency Of