Recombinant Human Acyl-Coenzyme A Dehydrogenase, C-2 to C-3 | MyBioSource MBS143508 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Human Acyl-Coenzyme A Dehydrogenase, C-2 to C-3

catalog :

MBS143508

quantity :

0.002 mg

price :

140 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS143508

products type :

Recombinant Protein

products full name :

Recombinant Human Acyl-Coenzyme A Dehydrogenase, C-2 to C-3

products short name :

Acyl-Coenzyme A Dehydrogenase

products name syn :

ACADS Human; Acyl-Coenzyme A Dehydrogenase C-2 to C-3 Human Recombinant; ACAD3; SCAD; EC 1.3.99.2; Short-chain specific acyl-CoA dehydrogenase; mitochondrial; Butyryl-CoA dehydrogenase; ACADS

other names :

short-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1; 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

products gene name :

ACADS

other gene names :

ACADS; ACADS; SCAD; ACAD3; SCAD

uniprot entry name :

ACADS_HUMAN

host :

E Coli

sequence length :

412

sequence :

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.

purity :

Greater than 95.0% as determined by SDS-PAGE.

form :

The protein contains 20mM Tris buffer pH-8, 1mM DTT, 20% glycerol and 100mM NaCl. Sterile filtered colorless solution.

storage stability :

Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.

products categories :

ENZYMES; Enzymes; Dehydrogenase

products description :

Description: ACADS Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 409 amino acids (25-412 a.a.) and having a molecular mass of 44 kDa. The ACADS protein is fused to a 20 amino acid His Tag at N-terminus and purified by standard chromatogrpahy techniques. Introduction: ACADS is a tetrameric mitochondrial flavoprotein, which is part of the acyl-CoA dehydrogenase family. ACADS catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in ACADS have been associated with Short Chain Acyl-CoA Dehydrogenase Deficiency.

ncbi gi num :

4557233

ncbi acc num :

NP_000008.1

ncbi gb acc num :

NM_000017.3

uniprot acc num :

P16219

ncbi mol weight :

44,297 Da

ncbi pathways :

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)

ncbi summary :

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]

uniprot summary :

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: EC 1.3.8.1; Lipid Metabolism - fatty acid; Carbohydrate Metabolism - butanoate; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase. 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; butyrate catabolic process; protein homotetramerization. Disease: Acyl-coa Dehydrogenase, Short-chain, Deficiency Of

size1 :

0.002 mg

price1 :

140 USD

size2 :

0.01 mg

price2 :

205

size3 :

1 mg

price3 :

5015

more info or order :

MyBioSource product webpage