Overview
Product nameACADSB protein (His-DHFR tag)See all ACADSB proteins and peptides ...
Protein descriptionRecombinant fragment of Human ACADSB (amino acids 180-402) with N terminal His-DHFR tag; 24.3kDa calculated without tag (UniProt ID = P45954).
Uniprot accessionP45954
Molecular weight24.300kDa
Protein length223 amino acids
Expression hostE. coli
Properties
Purification notesPurity is 90% by SDS-PAGE.
E.C. Number1.3.99.-
FormLyophilised:Reconstitute with water to desired concentration
Storage instructionsShipped at 4°C. Store at -20ºC.
Storage bufferConstituents: 0.32% Tris HCl, 0.58% Sodium chloride
Concentration information loading...
Research Areas
Applications
Our Abpromise guarantee covers the use of
ab127117
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 dilution. |
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 (His-DHFR tag) (ab127117)
ab127117
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"
