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货号： bs-23057R-FITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-23057R-FITC
- 英文名称
- Anti-ACADS/FITC
- 中文名称
- FITC标记的酰基辅酶A脱氢酶短链抗体
- 别 名
- ACAD3; Acyl Coenzyme A dehydrogenase, C2 to C3 short chain; Acyl-CoA dehydrogenase, C2 to C3 short chain; Acyl-CoA dehydrogenase, short chain; Acyl-Coenzyme A dehydrogenase, short chain; AI196007; Bcd-1; Bcd1; Butyryl CoA dehydrogenase; EC 1.3.99.2; SCAD; Short chain acyl CoA dehydrogenase; Short-chain specific acyl-CoA dehydrogenase, mitochondrial; Unsaturated acyl CoA reductase; ACADS_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 免疫学 转录调节因子 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig, Cow,
- 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
- 45kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human ACADS
- 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:
ACADS is a homotetramer mitochondrial flavoprotein, which is a member of the acyl CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta oxidation pathway. Mutations in this gene have been associated with Short Chain Acyl CoA Dehydrogenase Deficiency.
Subcellular Location:
Mitochondrion matrix.
DISEASE:
Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD) [MIM:201470]. It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults.
Similarity:
Belongs to the acyl-CoA dehydrogenase family.
Database links:Entrez Gene: 35 Human
Entrez Gene: 11409 Mouse
Entrez Gene: 64304 Rat
Omim: 606885 Human
SwissProt: P16219 Human
SwissProt: Q07417 Mouse
SwissProt: P15651 Rat
Unigene: 507076 Human
Unigene: 18759 Mouse
Unigene: 1167 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.