FITC标记的间隙连接蛋白47抗体
产品名称： FITC标记的间隙连接蛋白47抗体
英文名称： Anti-GJC2/FITC
产品编号： HZ-11050R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-GJC2/FITC Conjugated antibody
FITC标记的间隙连接蛋白47抗体
| 产品编号 | bs-11050R-FITC |
| 英文名称 | Anti-GJC2/FITC |
| 中文名称 | FITC标记的间隙连接蛋白47抗体 |
| 别 名 | Connexin 46.6; Connexin 47; Connexin-46.6; Connexin-47; Connexin46.6; Connexin47; CX 46.6; Cx 47; Cx46.6; Cx47; CXG2_HUMAN; Gap junction alpha 12 protein; Gap junction alpha-12 protein; Gap junction gamma 2 protein; Gap junction gamma-2 protein; Gap junction protein alpha 12 47kDa; Gap junction protein gamma 2 47kDa; GAP JUNCTION PROTEIN, 47-KD; gap junction protein, gamma 12, 47kDa; gap junction protein, gamma 2, 47kDa; GJA 12; GJA12; GJC 2; Gjc2; HLD 2; HLD2; PMLDAR; SPG44; CXG2_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 神经生物学 信号转导 细胞骨架 细胞外基质 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, Sheep, Guinea Pig, |
| 产品应用 | ICC=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 | 47kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human GJC2/Connexin 47 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | 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. |
| 产品介绍 | background: This gene encodes a gap junction protein. Gap junction proteins are members of a large family of homologous connexins and comprise 4 transmembrane, 2 extracellular, and 3 cytoplasmic domains. This gene plays a key role in central myelination and is involved in peripheral myelination in humans. Defects in this gene are the cause of autosomal recessive Pelizaeus-Merzbacher-like disease-1. [provided by RefSeq, Jul 2008] 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 role in myelination in central and peripheral nervous systems. Subunit: A connexon is composed of a hexamer of connexins. Interacts with TJP1 (By similarity). Subcellular Location: Cell membrane. Cell junction; gap junction. Tissue Specificity: Expressed in central nervous system, in sciatic nerve and sural nerve. Also detected in skeletal muscles. DISEASE: Defects in GJC2 are the cause of leukodystrophy hypomyelinating type 2 (HLD2) ; also known as Pelizaeus-Merzbacher-like disease autosomal recessive type 1. HLD2 is an autosomal recessive hypomyelinating leukodystrophy characterized by nystagmus, impaired motor development, ataxia, choreoathetotic movements, dysarthria and progressive spasticity. Defects in GJC2 are the cause of spastic paraplegia autosomal recessive type 44 (SPG44). A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. Similarity: Belongs to the connexin family. Gamma-type subfamily. Database links: Entrez Gene: 57165 Human Entrez Gene: 118454 Mouse Entrez Gene: 497913 Rat Omim: 608803 Human SwissProt: Q5T442 Human SwissProt: Q8BQU6 Mouse SwissProt: Q80XF7 Rat Unigene: 100072 Human Unigene: 40016 Mouse Unigene: 203000 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码间隙连接蛋白。间隙连接蛋白是一大家族同源连接蛋白的成员，包括4个跨膜、2个胞外和3个胞质结构域。该基因在中枢髓鞘形成中起关键作用，并参与人外周髓鞘形成。该基因的缺陷是常染色体隐性Pelizaeus Merzbacher样疾病的原因之一。[ RefSeq，JUL 2008 ]