Overview
Product nameACADSB proteinSee all ACADSB proteins and peptides ...
Protein descriptionRecombinant full length mature Human ACADSB (amino acids 34-432), fused to an N terminal His tag; 424 aa, 46.4 kDa. (NP_001600; P45954).
Uniprot accessionP45954
Molecular weight46.400kDa inclusive of tags
Protein length399 amino acids
Expression hostE. coli
Properties
Purity>90% by SDS-PAGE
Purification notesab130042 is purified using conventional chromatography techniques.
E.C. Number1.3.99.
FormLiquid
Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 8.00
Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride
Concentration information loading...
Sequence notesMGSSHHHHHH 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
Research Areas
Applications
Our Abpromise guarantee covers the use of
ab130042
in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Application
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Notes |
| SDS-PAGE |
SDS-PAGE: Use at an assay dependent concentration. |
Protein info
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Alternative names
2 MEBCAD2 methyl branched chain acyl CoA dehydrogenase2 methylbutyryl CoA dehydrogenase
2 methylbutyryl coenzyme A dehydrogenase2-MEBCAD2-methyl branched chain acyl-CoA dehydrogenase2-methylbutyryl-CoA dehydrogenase2-methylbutyryl-coenzyme A dehydrogenaseACAD7ACADSBACDSB_HUMANacyl CoA dehydrogenase, short/branched chainacyl Coenzyme A dehydrogenase short branched chainmitochondrialOTTHUMP00000020685OTTHUMP00000046795SBCADShort/branched chain specific acyl-CoA dehydrogenaseshort/branched chain specific acyl-CoA dehydrogenase, mitochondrial
see all
FunctionHas greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
Tissue specificityUbiquitous.
PathwayLipid metabolism; mitochondrial fatty acid beta-oxidation.
Involvement in diseaseDefects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD) [MIM:610006]; also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2-methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features.
Sequence similaritiesBelongs to the acyl-CoA dehydrogenase family.
Cellular localizationMitochondrion matrix.
References for ACADSB protein (ab130042)
ab130042
has not yet been referenced specifically in any publications.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"
SDS-PAGE - ACADSB protein (ab130042)15% SDS-PAGE showing ab130042 (3 µg).