TCTN1抗体

TCTN1抗体经ICC, IHC严格验证,品质保证.适用于多种种属反应性,被多篇文献引用并有用户反馈信息.确保特异性!产品具有以下特点:

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产品编号xy-12319R

英文名称TCTN1

中文名称结构蛋白家族1抗体

别    名TCTN1; TECT1_HUMAN; Tectonic-1.  

说 明 书100ul  200ul

研究领域细胞生物  **学  神经生物学  

抗体来源Rabbit

克隆类型Polyclonal

交叉反应 Human, Mouse, Rat, Pig, Cow, Sheep,

产品应用WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）

not yet tested in other applications.

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

分 子 量61kDa

细胞定位分泌型蛋白

性    状Lyophilized or Liquid

浓    度1mg/1ml

免 疫 原KLH conjugated synthetic peptide derived from human TCTN1

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.

PubMedPubMed

产品介绍background:

Regulator of Hedgehog (Hh), required for both activation and inhibition of the Hh pathway in the patterning of the neural tube. During neural tube development, it is required for formation of the most ventral cell types and for full Hh pathway activation. Functions in Hh signal transduction to fully activate the pathway in the presence of high Hh levels and to repress the pathway in the absence of Hh signals. Modulates Hh signal transduction downstream of SMO and RAB23.

Function:

Component of the tectonic-like complex, a complex localized at the transition zone of primary cilia and acting as a barrier that prevents diffusion of transmembrane proteins between the cilia and plasma membranes. Regulator of Hedgehog (Hh), required for both activation and inhibition of the Hh pathway in the patterning of the neural tube. During neural tube development, it is required for formation of the most ventral cell types and for full Hh pathway activation. Functions in Hh signal transduction to fully activate the pathway in the presence of high Hh levels and to repress the pathway in the absence of Hh signals. Modulates Hh signal transduction downstream of SMO and RAB23 (By similarity).

Subunit:

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

Subcellular Location:

Secreted.

DISEASE:

Defects in TCTN1 are the cause of Joubert syndrome type 13 (JBTS13) [MIM:614173]. JBTS13 is a disorder presenting with cerebellar ataxia, oculomotor apraxia, hypotonia, neonatal breathing abnormalities and psychomotor delay. Neuroradiologically, it is characterized by cerebellar vermian hypoplasia/aplasia, thickened and reoriented superior cerebellar peduncles, and an abnormally large interpeduncular fossa, giving the appearance of a molar tooth on transaxial slices (molar tooth sign). Additional variable features include retinal dystrophy and renal disease.

Similarity:

Belongs to the tectonic family.

Gene ID:

79600