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Rabbit Anti-Connexin 43/BF647 Conjugated antibody (bs-0651R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-0651R-BF647
英文名稱 Rabbit Anti-Connexin 43/BF647 Conjugated antibody
中文名稱 BF647標記的間隙連接蛋白43抗體
別    名 Connexin 43; connexin43; Connexin43v; Cx 43v; CX43; CX 43; CX-43; DFNB38; Gap junction 43 kDa heart protein;Connexin-43; Gap junction alpha 1 protein; Gap junction protein alpha 1 43kDa (connexin 43); Gap junction protein alpha 1 43kDa; Gap junction protein alpha like; GJA 1; GJA1; GJA-1; GJAL; HGNC:4282; HGNC:8112; Oculodentodigital dysplasia; ODD; ODOD; SDTY3; Syndactyly type III; CXA1_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  免疫學  轉錄調節因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat, Dog,  (predicted: Chicken, Cow, )
產品應用 Flow-Cyt=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 42kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Connexin-43 (211-260aa)
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.
產品介紹 background:
This gene 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 encoded 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. A related intronless pseudogene has been mapped to chromosome 5. Mutations in this gene have been associated with oculodentodigital dysplasia and heart malformations. [provided by RefSeq].

Function:
Gap junction protein that acts as a regulator of bladder capacity. A 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. Negative regulator of bladder functional capacity: acts by enhancing intercellular electrical and chemical transmission, thus sensitizing bladder muscles to cholinergic neural stimuli and causing them to contract.

Subunit:
A connexon is composed of a hexamer of connexins. Interacts (via C-terminus) with TJP1. Interacts (via C-terminus) with SRC (via SH3 domain). Interacts with UBQLN4. Interacts with SGSM3. Interacts with KIAA1432/CIP150. Interacts with CNST and CSNK1D.

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

Tissue Specificity:
Expressed in the heart and fetal cochlea.

Post-translational modifications:
Phosphorylated at Ser-368 by PRKCG; phosphorylation induces disassembly of gap junction plaques and inhibition of gap junction activity. Phosphorylation at Ser-325, Ser-328 and Ser-330 by CK1 modulates gap junction assembly.

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.

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

Database links:

Entrez Gene: 2697 Human

Entrez Gene: 14609 Mouse

Entrez Gene: 24392 Rat

Omim: 121014 Human

SwissProt: P17302 Human

SwissProt: P23242 Mouse

SwissProt: P08050 Rat

Unigene: 74471 Human




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

間隙連接蛋白-43(Gap junction alpha-1 protein; GJA-1; (Vascular smooth muscle connexin-43))是構成細胞間的通道,小分子成份可以借此在細胞間擴散。Connexin-43也是心肌縫隙連接的主要蛋白之一。
此外,星形細胞、成纖維細胞、平滑肌和腎等組織也有表達Connexin 43.
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