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货号： bs-19910R-PE-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19910R-PE-Cy3
- 英文名称
- Anti-PCCB/PE-Cy3
- 中文名称
- PE-Cy3标记的丙酰辅酶A羧化酶β链抗体
- 别 名
- DKFZp451E113; PCCase subunit beta; pccB; PCCB_HUMAN; pccBC Complementation group; Propanoyl CoA:carbon dioxide ligase subunit beta; Propanoyl-CoA:carbon dioxide ligase subunit beta; Propionyl CoA carboxylase beta chain, mitochondrial; propionyl Coenzyme A carboxylase, beta polypeptide; Propionyl-CoA carboxylase , beta subunit; Propionyl-CoA carboxylase beta chain, mitochondrial; R74805.
- 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 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
- 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
- 58kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PCCB
- 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:
The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]
Subunit:
Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits.
Subcellular Location:
Mitochondrion matrix.
DISEASE:
Defects in PCCB are the cause of propionic acidemia type II (PA-2) [MIM:606054]. PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein.
Similarity:
Belongs to the AccD/PCCB family.
Contains 1 carboxyltransferase domain.
Database links:Entrez Gene: 5096Human
Entrez Gene: 66904Mouse
Entrez Gene: 24624Rat
Omim: 232050Human
SwissProt: Q2TBR0Cow
SwissProt: P05166Human
SwissProt: Q99MN9Mouse
SwissProt: P79384Pig
SwissProt: P07633Rat
Unigene: 63788Human
Unigene: 335385Mouse
Unigene: 9732Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.