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Rabbit Anti-TCTN2/Gold Conjugated antibody (bs-12320R-Gold)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-12320R-Gold
英文名称 Anti-TCTN2/Gold
中文名称 胶体金标记的结构蛋白家族2抗体
别    名 C12orf38; FLJ12975; MKS8; OTTHUMP00000239215; OTTHUMP00000239216; Tctn2; TECT2; TECT2_HUMAN; Tectonic family member 2; Tectonic-2.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
研究领域 细胞生物  免疫学  发育生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 74kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from Human TCTN2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 background:
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

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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