protein

ACADS, 25-412aa, Human, His tag, E Coli

MBS203517

0.05 mg

375 USD

Recombinant Protein

ACADS, 25-412aa, Human, His tag, E Coli

ACADS

ACAD3; SCAD; Acyl-Coenzyme A dehydrogenase Acyl Coenzyme A dehydrogenase; C2 to C3 short chain; Acyl-CoA dehydrogenase; C2 to C3 short chain; Acyl-CoA dehydrogenase; short chain; Acyl-Coenzyme A dehydrogenase; short chain; AI196007; Bcd-1; Bcd1; Butyryl CoA dehydrogenase; EC 1.3.99.2; Short chain acyl CoA dehydrogenase; unsaturated acyl CoA reductase; Short-chain specific acyl-CoA dehydrogenase; mitochondrial.

Acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain; Short-chain specific acyl-CoA dehydrogenase, mitochondrial; short-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain; butyryl-CoA dehydrogenase; mitochondrial short-chain specific acyl-CoA dehydrogenase; unsaturated acyl-CoA reductase; acyl-CoA dehydrogenase, C-2 to C-3 short chain; Butyryl-CoA dehydrogenase

ACADS

ACADS; ACADS; SCAD; ACAD3; SCAD

ACADS_HUMAN

412

MGSSHHHHHH SSGLVPRGSH MLHTIYQSVE LPETHQMLLQ TCRDFAEKEL FPIAAQVDKE HLFPAAQVKK MGGLGLLAMD VPEELGGAGL DYLAYAIAME EISRGCASTG VIMSVNNSLY LGPILKFGSK EQKQAWVTPF TSGDKIGCFA LSEPGNGSDA GAASTTARAE GDSWVLNGTK AWITNAWEAS AAVVFASTDR ALQNKSISAF LVPMPTPGLT LGKKEDKLGI RGSSTANLIF EDCRIPKDSI LGEPGMGFKI AMQTLDMGRI GIASQALGIA QTALDCAVNY AENRMAFGAP LTKLQVIQFK LADMALALES ARLLTWRAAM LKDNKKPFIK EAAMAKLAAS EAATAISHQA IQILGGMGYV TEMPAERHYR DARITEIYEG TSEIQRLVIA GHLLRSYRS

> 95% by SDS - PAGE

Liquid. In 20 mM Tris-HCl buffer (pH8.0) containing 1mM DTT 0.1 M Nacl, and 20% glycerol

0.5 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

Redox Proteins

ACADS (Acyl-Coenzyme A dehydrogenase), also known as SCAD or ACAD3, is a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations of ACADS have been associated with fatty acid oxidation defects and metabolic diseases such as short-chain acyl-CoA dehydrogenase deficiency (SCAD deficiency). Recombinant human ACADS protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.

McAndrew RP., et al. (2008) J Biol Chem. 283(14):9435-43. Tafti M., et al. (2003) Nat Genet. 34(3):320-5.

19684166

AAH25963

44.0 kDa (409aa), confirmed by MALDI-TOF.

Beta Oxidation Of Butanoyl-CoA To Acetyl-CoA Pathway (106130); Beta Oxidation Of Hexanoyl-CoA To Butanoyl-CoA Pathway (106129); Butanoate Metabolism Pathway (83007); Butanoate Metabolism Pathway (391); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Fatty Acid Beta Oxidation Pathway (198865); Fatty Acid Degradation Pathway (82935); Fatty Acid Degradation Pathway (296); Fatty Acid Metabolism Pathway (868084)

This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with short-chain acyl-CoA dehydrogenase (SCAD) deficiency. Alternative splicing results in two variants which encode different isoforms. [provided by RefSeq, Oct 2014]

ACADS: Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD). It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults. Belongs to the acyl-CoA dehydrogenase family. Protein type: Lipid Metabolism - fatty acid; Oxidoreductase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.3.8.1; Carbohydrate Metabolism - butanoate. Chromosomal Location of Human Ortholog: 12q24.31. Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; nucleus. Molecular Function: acyl-CoA dehydrogenase activity; butyryl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding. Biological Process: response to starvation; fatty acid beta-oxidation; response to glucocorticoid stimulus; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; protein homotetramerization; butyrate catabolic process. Disease: Acyl-coa Dehydrogenase, Short-chain, Deficiency Of

0.05 mg

375 USD

0.25 mg

925