Recombinant Human SBCAD protein (ab130042)
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Overview
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Product name
Recombinant Human SBCAD protein
See all SBCAD proteins and peptides -
Protein length
Full length protein
Description
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Nature
Recombinant -
Source
Escherichia coli -
Amino Acid Sequence
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Accession
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Species
Human -
Sequence
MGSSHHHHHH 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 weight
46 kDa including tags -
Amino acids
34 to 432 -
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 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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Applications
SDS-PAGE
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Purity
> 90 % SDS-PAGE.
ab130042 is purified using conventional chromatography techniques. -
Form
Liquid -
Additional notes
Previously labelled as ACADSB.
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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: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride
General Info
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Alternative names
- 2 MEBCAD
- 2 methyl branched chain acyl CoA dehydrogenase
- 2 methylbutyryl CoA dehydrogenase
see all -
Function
Has 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 specificity
Ubiquitous. -
Pathway
Lipid metabolism; mitochondrial fatty acid beta-oxidation. -
Involvement in disease
Defects 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 similarities
Belongs to the acyl-CoA dehydrogenase family. -
Cellular localization
Mitochondrion matrix. - Information by UniProt
Images
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SDS-PAGE - Recombinant Human SBCAD protein (ab130042)15% SDS-PAGE showing ab130042 (3 µg).
Datasheets and documents
References
ab130042 has not yet been referenced specifically in any publications.
