Recombinant Human ACADVL/VLCAD protein (ab98234)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE
Description
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Product name
Recombinant Human ACADVL/VLCAD protein -
Purity
> 90 % SDS-PAGE.
ab98234 was purified using conventional chromatography techniques. -
Expression system
Escherichia coli -
Accession
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Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
MGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDALTRKKPAKAE SKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDP AKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGM HDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTD PATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPS ENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQF GEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILR LFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGL SLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLA DGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF -
Predicted molecular weight
69 kDa including tags -
Amino acids
41 to 655 -
Tags
His tag N-Terminus
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Associated products
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Related Products
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.
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Applications
SDS-PAGE
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Form
Liquid -
Additional notes
Previously labelled as ACADVL.
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Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 8.00
Constituents: 0.0154% DTT, 0.316% Tris HCl, 0.0292% EDTA, 10% Glycerol (glycerin, glycerine), 0.58% Sodium chloride
General Info
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Alternative names
- ACAD 6
- ACAD6
- ACADV_HUMAN
see all -
Function
Active 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. -
Pathway
Lipid metabolism; mitochondrial fatty acid beta-oxidation. -
Involvement in disease
Defects 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 similarities
Belongs to the acyl-CoA dehydrogenase family. -
Cellular localization
Mitochondrion inner membrane. - Information by UniProt
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab98234 has not yet been referenced specifically in any publications.