| 产品编号 | bsm-51507M |
| 英文名称 | GJC2 |
| 中文名称 | 间隙连接蛋白47单克隆抗体 |
| 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; HLD 2; HLD2; PMLDAR; SPG44; CXG2_HUMAN. |
| 研究领域 | 神经生物学 信号转导 骨髓细胞 细胞外基质 |
| 抗体来源 | Mouse |
| 克隆类型 | Monoclonal |
| 克 隆 号 | D4F12 |
| 交叉反应 | Human |
| 产品应用 | WB=1:500-1000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 47kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human GJC2: 1-100/439 |
| 亚 型 | IgM |
| 纯化方法 | affinity purified by Protein G |
| 缓 冲 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
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. 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. SWISS: Q5T442 Gene ID: 57165 Database links: Entrez Gene: 57165 Human SwissProt: Q5T442 Human |
| 产品图片 |
Sample:
Lane 1: A549 cell lysates Primary: Anti-GJC2 (bsm-51507M) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Mouse IgG at 1/20000 dilution Predicted band size: 47 kD Observed band size: 47 kD |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
