Overview

  • Product nameHuman ACADVL full length protein
  • DescriptionHuman ACADVL full length protein

Description

  • NatureRecombinant
  • SourceE. coli
  • Amino Acid Sequence
    • AccessionP49748
    • SpeciesHuman
    • SequenceMGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDALTRKKPAKAE SKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDP AKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGM HDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTD PATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPS ENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQF GEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILR LFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGL SLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLA DGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF
    • Molecular weight69 kDa including tags
    • Amino acids41 to 655
    • TagsHis tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab98234 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.
    ab98234 was 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.

    Preservative: None
    Constituents: 10% Glycerol, 0.1M Sodium chloride, 20mM Tris HCl, 1mM EDTA, 1mM DTT, pH 8.0

General info

  • Alternative names
    • ACAD 6
    • ACAD6
    • ACADV_HUMAN
    • Acadvl
    • Acyl CoA dehydrogenase very long chain
    • Acyl Coenzyme A dehydrogenase very long chain
    • LCACD
    • mitochondrial
    • Very long chain specific acyl CoA dehydrogenase
    • Very long chain specific acyl CoA dehydrogenase mitochondrial
    • Very long-chain specific acyl-CoA dehydrogenase
    • VLCAD
    see all
  • FunctionActive toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
  • PathwayLipid metabolism; mitochondrial fatty acid beta-oxidation.
  • Involvement in diseaseDefects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:201475]. ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.
  • Sequence similaritiesBelongs to the acyl-CoA dehydrogenase family.
  • Cellular localizationMitochondrion inner membrane.
  • Target information above from: UniProt accession P49748 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt

Human ACADVL full length protein images

  • 15% SDS-PAGE analysis of 3µg ab98234

References for Human ACADVL full length protein (ab98234)

ab98234 has not yet been referenced specifically in any publications.

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