結(jié)構(gòu)蛋白家族2抗體說(shuō)明書 英文名稱  Anti-TCTN2

中文名稱  結(jié)構(gòu)蛋白家族2抗體說(shuō)明書 

別    名 C12orf38; FLJ12975; MKS8; OTTHUMP00000239215; OTTHUMP00000239216; Tctn2; TECT2; TECT2_HUMAN; Tectonic family member 2; Tectonic-2.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來(lái)源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat

產(chǎn)品類型   一抗  

研究領(lǐng)域    細(xì)胞生物 免疫學(xué) 發(fā)育生物學(xué)

蛋白分子量  predicted molecular weight: 74kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from Human TCTN2

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

結(jié)構(gòu)蛋白家族2抗體說(shuō)明書 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 ICC=1:100-500 IF=1:100-500

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

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

產(chǎn)品介紹 Defects in TCTN2 are the cause of Meckel syndrome type 8 (MKS8) [MIM:613885]. A disorder characterized by a combination of renal cysts and variably associated features including developmental anomalies of the central nervous system (typically encephalocele), hepatic ductal dysplasia and cysts, and polydactyly.

Function : Component of the tectonic-like complex, a complexlocalized at the transition zone of primary cilia and acting as abarrier that prevents diffusion of transmembrane proteins betweenthe cilia and plasma membranes. Required for hedgehog signalingtransduction (By similarity).

Subunit : Part of the tectonic-like complex (also named B9 complex)(By similarity).

Subcellular Location : Membrane; Single-pass type I membraneprotein (Potential). Cytoplasm, cytoskeleton, cilium basal body (Bysimilarity). Note=Localizes at the transition zone, a regionbetween the basal body and the ciliary axoneme (By similarity).

DISEASE : Defects in TCTN2 are the cause of Meckel syndrome type 8(MKS8) [MIM:613885]. A disorder characterized by a combination ofrenal cysts and variably associated features includingdevelopmental anomalies of the central nervous system (typicallyencephalocele), hepatic ductal dysplasia and cysts, andpolydactyly.

Note=Defects in TCTN2 may be a cause of Joubert syndrome,a disorder presenting with cerebellar ataxia, oculomotor apraxia,hypotonia, neonatal breathing abnormalities and psychomotor delay.Neuroradiologically, it is characterized by cerebellar vermianhypoplasia/aplasia, thickened and reoriented superior cerebellarpeduncles, and an abnormally large interpeduncular fossa, givingthe appearance of a molar tooth on transaxial slices (molar toothsign). Additional variable features include retinal dystrophy andrenal disease.

Similarity : Belongs to the tectonic family.

Database links : UniProtKB/Swiss-Prot: Q96GX1.1

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