catalog number :
MBS203704
products type :
Recombinant Protein
products full name :
ACADM, 26-421aa, Human, His tag, E Coli
products short name :
ACADM
products name syn :
ACAD1; CAD; MCADH; Medium-chain specific acyl-CoA dehydrogenase; ACAD 1; Acyl Coenzyme A Dehydrogenase; Acyl coenzyme A dehydrogenase C 4 to C 12 straight chain; C4 to C12 Straight Chain; FLJ18227; FLJ93013; FLJ99884; MCAD; Medium chain acyl CoA dehydrogenase; Medium chain fatty acyl CoA dehydrogenase; Medium chain specific acyl CoA dehydrogenase; Medium chain specific acyl CoA dehydrogenase mitochondrial
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 :
> 90% by SDS - PAGE
form :
Liquid. 20mM Tris-HCl buffer (pH8.0) containing 20% glycerol 0.1M NaCl
concentration :
0.5 mg/ml (determined by Bradford assay)
storage stability :
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.
tested application :
SDS-PAGE
other info1 :
Antigen Species: Human. Tag: His-tag
other info2 :
Expression System: E Coli
products categories :
Enzymes & Proteases
products description :
ACADM, also known as acyl-Coenzyme A dehydrogenase is an enzyme that is important for breaking down (degrading) a certain group of fats called medium-chain fatty acids. It is essential for converting these particular fatty acids to energy, especially during periods without food (fasting). This protein functions in mitochondria, the energy-producing centers within cells. It is found in the mitochondria of several types of tissues, particularly the liver. Recombinant human ACADM protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques
products references :
Gregersen N., et al. (2001) Hum Mutat. 18(3):169-89.
ncbi gb acc num :
NM_000016.5
ncbi mol weight :
45.9 kDa (417aa) confirmed by MALDI-TOF
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: Carbohydrate Metabolism - propanoate; EC 1.3.8.7; Mitochondrial; Other Amino Acids Metabolism - beta-alanine; Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase. 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