Human ACADSB full length protein (ab130042)

Overview

Description

  • NatureRecombinant
  • SourceE. coli
  • Amino Acid Sequence
    • AccessionP45954
    • SpeciesHuman
    • SequenceMGSSHHHHHH 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
    • Molecular weight46 kDa including tags
    • Amino acids34 to 432
    • TagsHis tag N-Terminus

Specifications

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.

  • Applications

    SDS-PAGE

  • Purity>90% by SDS-PAGE.
    ab130042 is purified using conventional chromatography techniques.
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride

General info

  • Alternative names
    • 2 MEBCAD
    • 2 methyl branched chain acyl CoA dehydrogenase
    • 2 methylbutyryl CoA dehydrogenase
    • 2 methylbutyryl coenzyme A dehydrogenase
    • 2-MEBCAD
    • 2-methyl branched chain acyl-CoA dehydrogenase
    • 2-methylbutyryl-CoA dehydrogenase
    • 2-methylbutyryl-coenzyme A dehydrogenase
    • ACAD7
    • ACADSB
    • ACDSB_HUMAN
    • acyl CoA dehydrogenase, short/branched chain
    • acyl Coenzyme A dehydrogenase short branched chain
    • mitochondrial
    • OTTHUMP00000020685
    • OTTHUMP00000046795
    • SBCAD
    • Short/branched chain specific acyl-CoA dehydrogenase
    • short/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.
  • Information by UniProt

Human ACADSB full length protein images

  • 15% SDS-PAGE showing ab130042 (3 µg).

References for Human ACADSB full length protein (ab130042)

ab130042 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab130042.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"