CC2D2A兔多克隆 | MyBioSource MBS767569 产品信息

产品简要

公司名称 :

MyBioSource

产品类型 :

抗体

产品名称 :

CC2D2A兔多克隆

MBS767569

规格 :

0.1毫克

价格 :

265美元

克隆性 :

多克隆

宿主 :

兔

共轭标签 :

未共轭

反应物种 :

人类, 小鼠

应用 :

免疫印迹, 酶联免疫吸附测定, 免疫组化

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图像

图像 1 :

Immunohistochemistry of paraffin-embedded human lung cancer slide using MBS767569 ( CC2D2A Antibody) at dilution of 1:50

图像 2 :

HEK-293 cells were subjected to SDS PAGE followed by western blot with MBS767569 (CC2D2A antibody) at dilution of 1:500

产品信息

目录号 :

MBS767569

产品类型 :

抗体

产品全称 :

CC2D2A兔多克隆

产品简称 :

[CC2D2A]

产品名称同义词 :

[KIAA1345, CC2D2A, JBTS9, MKS6]

其他名称 :

[coiled-coil and C2 domain-containing protein 2A isoform a; Coiled-coil and C2 domain-containing protein 2A; coiled-coil and C2 domain-containing protein 2A; coiled-coil and C2 domain containing 2A]

产品基因名称 :

[CC2D2A]

其他基因名称 :

[CC2D2A;CC2D2A;MKS6;JBTS9;KIAA1345]

UniProt数据库进入名 :

C2D2A_HUMAN

克隆性 :

多克隆

抗体亚型 :

IgG

宿主 :

兔

反应物种 :

人类, 小鼠

序列长度 :

1620

纯度 :

Purity: >=95% as determined by SDS-PAGE. Purification: Immunogen Affinity Purified

形式 :

液体

储存稳定性 :

Store at -20°C for 24 months (Avoid repeated freeze / thaw cycles.)

检测过的应用 :

酶联免疫吸附测定, 免疫印迹, 免疫组化

应用笔记 :

WB: 1:500-1:1000。 IHC: 1:20-1:200

image1头 :

免疫组化

image2头 :

免疫印迹

其它信息1 :

Immunogen: Coiled-coil and C2 domain containing 2A. Buffer: PBS with 0.02% sodium azide and 50% glycerol pH 7.3

产品描述 :

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. Required for ciliogenesis and sonic hedgehog/SHH signaling (By similarity).

NCBI GI登录号 :

197209974

NCBI登录号 :

NP_001073991.2

NCBI基因登录号 :

NM_001080522.2

UniProt数据库登录号 :

Q9P2K1

NCBI分子量 :

190 kDa

NCBI信号通路 :

Anchoring Of The Basal Body To The Plasma Membrane Pathway (1268847); Assembly Of The Primary Cilium Pathway (1268846); Organelle Biogenesis And Maintenance Pathway (1268838)

NCBI总结 :

This gene encodes a coiled-coil and calcium binding domain protein that appears to play a critical role in cilia formation. Mutations in this gene cause Meckel syndrome type 6, as well as Joubert syndrome type 9. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2009]

UniProt数据库总结 :

CC2D2A: 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. Required for ciliogenesis and sonic hedgehog/SHH signaling. Defects in CC2D2A are the cause of Meckel syndrome type 6 (MKS6). MKS is an autosomal recessive 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. Defects in CC2D2A are the cause of Joubert syndrome type 9 (JBTS9). JBTS is an autosomal recessive 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. Defects in CC2D2A are a cause of COACH syndrome (COACHS). It is a disorder characterized by mental retardation, ataxia due to cerebellar hypoplasia, and hepatic fibrosis. Patients present the molar tooth sign, a midbrain- hindbrain malformation pathognomonic for Joubert syndrome and related disorders. Other features, such as coloboma and renal cysts, may be variable. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Cytoskeletal. Chromosomal Location of Human Ortholog: 4p15.32. Cellular Component: cytoskeleton; cytosol. Biological Process: axoneme biogenesis; camera-type eye development; cilium biogenesis; determination of left/right symmetry; heart development; neural tube closure; sensory cilium biogenesis; smoothened signaling pathway. Disease: Coach Syndrome; Joubert Syndrome 9; Meckel Syndrome, Type 6

尺寸1 :

0.1毫克

价格1 :

265美元

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公司信息