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Rabbit Anti-Connexin 26/Gold Conjugated antibody (bs-1715R-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-1715R-Gold
英文名称 Anti-Connexin 26/Gold
中文名称 胶体金标记的间隙连接蛋白26/GJB2抗体
别    名 cx26; DFNA3; DFNB1; HID; KID; NSRD1; Connexin26; Connexin-26; GJB2; PPK; CXB2_HUMAN.  
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 肿瘤  细胞生物  神经生物学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
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 26kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Connexin-26
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:
Gap junction channels consist of connexin protein subunits, which are encoded by a multigene family. GJBs (gap junction proteins or connexins) play crucial functional roles associated with these channels. Immunohistochemical staining of human cochlear cells demonstrated high levels of GJB2 expression. Mutations in GJB2 are associated with genetically derived hearing impairments, including autosomal recessive nonsyndromic deafness.

Function:
One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexons, through which materials of low MW diffuse from one cell to a neighboring cell. May play a critical role in the physiology of hearing by participating in the recycling of potassium to the cochlear endolymph.

Subcellular Location:
Cell membrane. Cell junction, gap junction.

Tissue Specificity:
Expressed in the heart and fetal cochlea.

Post-translational modifications:
Belongs to the connexin family. Beta-type (group I) subfamily.

DISEASE:
Defects in GJA1 are the cause of autosomal dominant oculodentodigital dysplasia (ODDD) [MIM:164200]; also known as oculodentoosseous dysplasia. ODDD is a highly penetrant syndrome presenting with craniofacial (ocular, nasal, dental) and limb dysmorphisms, spastic paraplegia, and neurodegeneration. Craniofacial anomalies tipically include a thin nose with hypoplastic alae nasi, small anteverted nares, prominent columnella, and microcephaly. Brittle nails and hair abnormalities of hypotrichosis and slow growth are present. Ocular defects include microphthalmia, microcornea, cataracts, glaucoma, and optic atrophy. Syndactyly type 3 and conductive deafness can occur in some cases. Cardiac abnormalities are observed in rare instances.
Defects in GJA1 are the cause of autosomal recessive oculodentodigital dysplasia (ODDD autosomal recessive) [MIM:257850].

Similarity:
Belongs to the connexin family. Alpha-type (group II) subfamily.

Database links:

Entrez Gene: 2706 Human

Entrez Gene: 14619 Mouse

Entrez Gene: 394266 Rat

Omim: 121011 Human

SwissProt: P29033 Human

SwissProt: Q00977 Mouse

SwissProt: P21994 Rat

Unigene: 524894 Human

Unigene: 714494 Human

Unigene: 390683 Mouse

Unigene: 219269 Rat



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

间隙连接(Gap junction, GJ)是普遍存在于相邻细胞间的细胞连接方式,是相邻细胞膜上的一种膜蛋白通道结构。间隙连接允许邻近细胞之间的离子,核苷酸和小的调节分子以及药物或它们的代谢物进行转运,细胞通过它所介导的细胞间连接通讯,进行细胞间信息和能量的传递,调控细胞的生长、分化和内环境的稳定,对维持机体的功能发挥重要作用.
间隙连接蛋白26目前主要用于肿瘤方面的研究。
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