Human GCDH Recombinant Protein (RPPB1680)
- SKU:
- RPPB1680
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- Q92947
- Research Area:
- Enzymes
Description
| Product Name: | Human GCDH Recombinant Protein |
| Product Code: | RPPB1680 |
| Size: | 10µg |
| Species: | Human |
| Target: | GCDH |
| Synonyms: | ACAD5, GCD, EC 1.3.99.7, GCDH, Glutaryl-Coenzyme A Dehydrogenase, glutaryl-CoA dehydrogenase. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile filtered colorless solution. |
| Formulation: | 0.5mg/ml solution containing 20mM Tris-HCl, pH-8, 5mM DTT, 0.2M NaCl & 20% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 90.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MRPEFDWQDP LVLEEQLTTD EILIRDTFRT YCQERLMPRI LLANRNEVFH REIISEMGEL GVLGPTIKGY GCAGVSSVAY GLLARELERV DSGYRSAMSV QSSLVMHPIY AYGSEEQRQK YLPQLAKGEL LGCFGLTEPN SGSDPSSMET RAHYNSSNKS YTLNGTKTWI TNSPMADLFV VWARCEDGCI RGFLLEKGMR GLSAPRIQGK FSLRASATGM IIMDGVEVPE ENVLPGASSL GGPFGCLNNA RYGIAWGVLG ASEFCLHTAR QYALDRMQFG VPLARNQLIQ KKLADMLTEI TLGLHACLQL GRLKDQDKAA PEMVSLLKRN NCGKALDIAR QARDMLGGNG ISDEYHVIRH AMNLEAVNTY EGTHDIHALI LGRAITGIQA FTASK |
GCDH is part of the acyl-CoA dehydrogenase family. GCDH is localized in the mitochondrial matrix as a homotetramer of 45-kD subunits. GCDH 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. GCDH uses electron transfer flavoprotein as its electron acceptor.
GCDH Recombinant Human produced in E.Coli is a single, non-glycosylated polypeptide chain containing 415 amino acids (45-438 a.a.) and having a molecular mass of 45.8 kDa. The GCDH is fused to 21 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.
| UniProt Protein Function: | GCDH: 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. Defects in GCDH are the cause of glutaric aciduria type 1 (GA1). GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Lipid Metabolism - fatty acid; Amino Acid Metabolism - tryptophan; Mitochondrial; Oxidoreductase; Amino Acid Metabolism - lysine degradation; EC 1.3.8.6 Chromosomal Location of Human Ortholog: 19p13.2 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:acyl-CoA binding; electron carrier activity; FAD binding; glutaryl-CoA dehydrogenase activity Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; lysine catabolic process Disease: Glutaric Acidemia I |
| NCBI Summary: | The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It 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. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013] |
| UniProt Code: | Q92947 |
| NCBI GenInfo Identifier: | 2492631 |
| NCBI Gene ID: | 2639 |
| NCBI Accession: | Q92947.1 |
| UniProt Secondary Accession: | Q92947,O14719, A8K2Z2, |
| UniProt Related Accession: | Q92947 |
| Molecular Weight: | 47,355 Da |
| NCBI Full Name: | Glutaryl-CoA dehydrogenase, mitochondrial |
| NCBI Synonym Full Names: | glutaryl-CoA dehydrogenase |
| NCBI Official Symbol: | GCDH�� |
| NCBI Official Synonym Symbols: | GCD; ACAD5�� |
| NCBI Protein Information: | glutaryl-CoA dehydrogenase, mitochondrial |
| UniProt Protein Name: | Glutaryl-CoA dehydrogenase, mitochondrial |
| UniProt Gene Name: | GCDH�� |
| UniProt Entry Name: | GCDH_HUMAN |
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