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Rabbit Anti-PRRT2/BF594 Conjugated antibody (bs-19430R-BF594)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-19430R-BF594
英文名稱(chēng) Rabbit Anti-PRRT2/BF594 Conjugated antibody
中文名稱(chēng) BF594標(biāo)記的富含脯氨酸跨膜蛋白2抗體
別    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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  跨膜蛋白  細(xì)胞膜蛋白  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Dog, Pig, Cow, Horse, )
產(chǎn)品應(yīng)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 35, 40 kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PRRT2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 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: 112476 Human

Entrez Gene: 69017 Mouse

Entrez Gene: 361651 Rat

Omim: 614386 Human

SwissProt: Q7Z6L0 Human

SwissProt: E9PUL5 Mouse

SwissProt: D3ZFB6 Rat

Unigene: 655071 Human

Unigene: 392047 Mouse

Unigene: 51543 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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