| Applications: | SDS-PAGE |
| Species Reactivity: | Human |
| MW: | 46.4 kDa (424aa) |
| Purity: | > 90% by SDS - PAGE |
| Description: | 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. |
| Further Information: | Sequence: 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 |
| Synonym: | Short/branched chain specific acyl-CoA dehydrogenase mitochondrial, Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial, 2-MEBCAD, ACAD7, SBCAD |
| Ascension Number: | P45954 |
| NCBI EntrezGene: | NP_001600 |
| Shipping Conditions: | Ship on cold packs |
| Storage: | Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles. |
| Usage: | Research use only |
