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Rabbit Anti-Connexin-32/BF555 Conjugated antibody (bs-1376R-BF555)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-1376R-BF555
英文名稱 Rabbit Anti-Connexin-32/BF555 Conjugated antibody
中文名稱 BF555標記的間隙連接蛋白32抗體
別    名 GJB1; Connexin-32; CX32; Connexin32; Connexin 32; Cx32; CXB1_HUMAN; Charcot Marie Tooth neuropathy X linked; CMTX 1; CMTX1; CX 32; GAP junction 28 kDa liver protein; Gap junction beta 1 protein; Gap junction beta-1 protein; Gap junction protein beta 1 32kD; Gap junction protein beta 1; Gap junction protein beta-1 32kD; GJB 1;CMTX; CMTX1.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 免疫學  神經(jīng)生物學  通道蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, )
產(chǎn)品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 32kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Connexin-32
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
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.
產(chǎn)品介紹 background:
Vascular smooth muscle connexin-32 is a member of the connexin gene family. The encoded protein is a component of gap junctions, which are composed of arrays of intercellular channels that provide a route for the diffusion of low molecular weight materials from cell to cell. The protein is the major protein of gap junctions in the heart that are thought to have a crucial role in the synchronized contraction of the heart and in embryonic development. Mutations in this gene have been associated with oculodentodigital dysplasia and heart malformations.

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.

Subunit:
A connexon is composed of a hexamer of connexins. Interacts with CNST.

Subcellular Location:
Cell membrane; Multi-pass membrane protein. Cell junction, gap junction.

DISEASE:
Defects in GJB1 are the cause of Charcot-Marie-Tooth disease X-linked type 1 (CMTX1) [MIM:302800]; also designated CMT-X. CMTX1 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy. CMTX1 has both demyelinating and axonal features. Central nervous system involvement may occur.
Defects in GJB1 may contribute to the phenotype of Dejerine-Sottas syndrome (DSS) [MIM:145900]; also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS is a severe degenerating neuropathy of the demyelinating Charcot-Marie-Tooth disease category, with onset by age 2 years. DSS is characterized by motor and sensory neuropathy with very slow nerve conduction velocities, increased cerebrospinal fluid protein concentrations, hypertrophic nerve changes, delayed age of walking as well as areflexia. There are both autosomal dominant and autosomal recessive forms of Dejerine-Sottas syndrome.

Similarity:
Belongs to the connexin family. Beta-type (group I) subfamily.

Database links:

Entrez Gene: 2705 Human

Entrez Gene: 14618 Mouse

Entrez Gene: 29584 Rat

Omim: 304040 Human

SwissProt: O18968 Cow

SwissProt: P08034 Human

SwissProt: P28230 Mouse

SwissProt: P08033 Rat

Unigene: 333303 Human

Unigene: 21198 Mouse

Unigene: 10444 Rat



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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