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Product name Recombinant Human GCDH protein
Protein length Full length protein Description
Source Escherichia coli
Amino Acid Sequence
Sequence MGSSHHHHHHSSGLVPRGSHMRPEFDWQDPLVLEEQLTTDEILIRDTFRT YCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAY GLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGEL LGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFV VWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPE ENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFG VPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRN NCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALI LGRAITGIQAFTASK
Molecular weight 46 kDa including tags
Amino acids 45 to 438
Tags His tag N-Terminus Specifications
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in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Purity > 90
% SDS-PAGE. ab98118 is purified by using conventional chromatography techniques.
Concentration information loading... Preparation and Storage
Stability and Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Constituents: 20% Glycerol, 5mM DTT, 200mM Sodium chloride, 20mM Tris HCl, pH 8
Function Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.
Tissue specificity Isoform 1 and isoform 2 are expressed in fibroblasts and liver.
Pathway Amino-acid metabolism; lysine degradation. Amino-acid metabolism; tryptophan metabolism.
Involvement in disease Defects in GCDH are the cause of glutaric aciduria type 1 (GA1) [MIM:231670]. GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.
Sequence similarities Belongs to the acyl-CoA dehydrogenase family.
Cellular localization Mitochondrion matrix.
Information by UniProt
Recombinant Human GCDH protein images
References for Recombinant Human GCDH protein (ab98118)
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"