protein

ACADSB, 34-432aa, Human, His tag, E Coli

MBS204623

0.05 mg

375 USD

Recombinant Protein

ACADSB, 34-432aa, Human, His tag, E Coli

ACADSB

Short/branched chain specific acyl-CoA dehydrogenase; mitochondrial; 2-MEBCAD; ACAD7; SBCAD

short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; 2-methyl branched chain acyl-CoA dehydrogenase; 2-methylbutyryl-coenzyme A dehydrogenase; acyl-Coenzyme A dehydrogenase, short/branched chain; acyl-CoA dehydrogenase, short/branched chain; 2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenase

ACADSB

ACADSB; ACADSB; ACAD7; SBCAD; 2-MEBCAD; SBCAD; 2-MEBCAD; 2-methylbutyryl-CoA dehydrogenase

ACDSB_HUMAN

432

MGSSHHHHHH SSGLVPRGSH MGSHMKSSQS EALLNITNNG IHFAPLQTFT DEEMMIKSSV KKFAQEQIAP LVSTMDENSK MEKSVIQGLF QQGLMGIEVD PEYGGTGASF LSTVLVIEEL AKVDASVAVF CEIQNTLINT LIRKHGTEEQ KATYLPQLTT EKVGSFCLSE AGAGSDSFAL KTRADKEGDY YVLNGSKMWI SSAEHAGLFL VMANVDPTIG YKGITSFLVD RDTPGLHIGK PENKLGLRAS STCPLTFENV KVPEANILGQ IGHGYKYAIG SLNEGRIGIA AQMLGLAQGC FDYTIPYIKE RIQFGKRLFD FQGLQHQVAH VATQLEAARL LTYNAARLLE AGKPFIKEAS MAKYYASEIA GQTTSKCIEW MGGVGYTKDY PVEKYFRDAK IGTIYEGASN IQLNTIAKHI DAEY

> 90% by SDS - PAGE

Liquid. In 20mM Tris-HCl buffer (pH 8.0) containing 0.1M NaCl, 10% glycerol,1mM DTT

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

Signal Transduction

Short/branched chain specific acyl-CoA dehydrogenase(ACADSB), is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. ACADSB catalyzes the degradation of L-isoleucine and has the highest affinity for (s)-2-methylbutyryl-CoA, isobutyryl-CoA and 2-methylhexanoyl-CoA as substrates. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), an autosomal recessive disorder characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Recombinant human ACADSB protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.

Arden K C., et al. (1995) Genomics. 25: 743-745. Korman S H., et al. (2005) Clin Chem. 51: 610-617.

4501859

NP_001600

NM_001609.3

46.4 kDa (424aa)

Branched-chain Amino Acid Catabolism Pathway 106179!!Fatty Acid Degradation Pathway 82935!!Fatty Acid Degradation Pathway 296!!Fatty Acid Metabolism Pathway 868084!!Fatty Acid Metabolism Pathway 878045!!Metabolic Pathways 132956!!Metabolism Pathway 477135!!Metabolism Of Amino Acids And Derivatives Pathway 106169!!Valine, Leucine And Isoleucine Degradation Pathway 82952!!Valine, Leucine And Isoleucine Degradation Pathway 316

Short/branched chain acyl-CoA dehydrogenase(ACADSB) is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. Substrate specificity is the primary characteristic used to define members of this gene family. The ACADSB gene product has the greatest activity towards the short branched chain acyl-CoA derivative, (S)-2-methylbutyryl-CoA, but also reacts significantly with other 2-methyl branched chain substrates and with short straight chain acyl-CoAs. The cDNA encodes for a mitochondrial precursor protein which is cleaved upon mitochondrial import and predicted to yield a mature peptide of approximately 43.7-KDa. [provided by RefSeq, Jul 2008]

0.05 mg

375 USD