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货号： bs-13312R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-13312R-PE-Cy7
- 英文名称
- Anti-GCDH/PE-Cy7
- 中文名称
- PE-Cy7标记的戊二酰辅酶A脱氢酶抗体
- 别 名
- ACAD5; EC 1.3.99.7; GCD; Gcdh; GCDH_HUMAN; Glutaryl CoA dehydrogenase, mitochondrial; Glutaryl Coenzyme A dehydrogenase; Glutaryl-CoA dehydrogenase; mitochondrial; MS781.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 神经生物学 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 43kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human GCDH
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze 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. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.
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.
Subunit:
Homotetramer.
Subcellular Location:
Mitochondrion matrix.
Tissue Specificity:
Isoform 1 and isoform 2 are expressed in fibroblasts and liver.
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.
Similarity:
Belongs to the acyl-CoA dehydrogenase family.
Database links:
UniProtKB/Swiss-Prot: Q92947.1
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.