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货号： bs-19430R-AP 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19430R-AP
- 英文名称
- Anti-PRRT2/AP
- 中文名称
- 碱性磷酸酶（AP）标记的富含脯氨酸跨膜蛋白2抗体
- 别 名
- interferon induced transmembrane protein domain containing 1; BFIC2; BFIS2; Dispanin subfamily B member 3; DSPB3; DYT10; EKD1; FLJ25513; ICCA; IFITMD1; interferon induced transmembrane protein domain containing 1; PKC; Proline rich transmembrane protein 2; Proline-rich transmembrane protein 2; PRRT2; PRRT2_HUMAN.
- 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 免疫学 神经生物学 跨膜蛋白 细胞膜蛋白
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Dog, Pig, Cow, Horse,
- 产品应用
- WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 35, 40 kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PRRT2
- 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:
This gene encodes a transmembrane protein containing a proline-rich domain in its N-terminal half. Studies in mice suggest that it is predominantly expressed in brain and spinal cord in embryonic and postnatal stages. Mutations in this gene are associated with episodic kinesigenic dyskinesia-1. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2012]
Subcellular Location:
Cell membrane. Cell junction >synapse.
DISEASE:
Episodic kinesigenic dyskinesia 1 (EKD1) [MIM:128200]: An autosomal dominant neurologic condition characterized by recurrent and brief attacks of abnormal involuntary movements, triggered by sudden voluntary movement. These attacks usually have onset during childhood or early adulthood and can involve dystonic postures, chorea, or athetosis. Note=The disease is caused by mutations affecting the gene represented in this entry. Disease-causing mutations that produce truncation of the C-terminus of the protein alter subcellular location, from plasma membrane to cytosplasm (PubMed:22101681).
Convulsions, familial infantile, with paroxysmal choreoathetosis (ICCA)
[MIM:602066]: A syndrome characterized by clinical features of benign familial infantile seizures and episodic kinesigenic dyskinesia. Benign familial infantile seizures is a disorder characterized by afebrile seizures occurring during the first year of life, without neurologic sequelae. Paroxysmal choreoathetosis is a disorder of involuntary movements characterized by attacks that occur spontaneously or are induced by a variety of stimuli. Note=The disease is caused by mutations affecting the gene represented in this entry.
Seizures, benign familial infantile 2 (BFIS2) [MIM:605751]: An autosomal dominant disorder in which afebrile seizures occur in clusters during the first year of life, without neurologic sequelae. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the CD225/Dispanin family.
Database links:Entrez Gene: 112476Human
Entrez Gene: 69017Mouse
Entrez Gene: 361651Rat
Omim: 614386Human
SwissProt: Q7Z6L0Human
SwissProt: E9PUL5Mouse
SwissProt: D3ZFB6Rat
Unigene: 655071Human
Unigene: 392047Mouse
Unigene: 51543Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.