GCDH Antibody, Biotin conjugated (PACO39521)
- SKU:
- PACO39521
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- Biotin
Frequently bought together:
Description
抗体名: | GCDH Antibody, Biotin conjugated (PACO39521) |
抗体コード: | PACO39521 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Glutaryl-CoA dehydrogenase, mitochondrial protein (45-300AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Biotin |
バックグラウンド: | 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. |
シノニム: | Glutaryl-CoA dehydrogenase, mitochondrial (GCD) (EC 1.3.8.6), GCDH |
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 |