间隙连接蛋白47抗体

间隙连接蛋白47抗体英文名称  Anti-GJC2/Connexin 47 

中文名称  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. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg     

纯化的抗体可通过不同的途径获取，有些间隙连接蛋白47抗体可通过下述方法制备或从商家购买。从商家购买的抗体，通常附有正确的储存方法。

1）工作液应在4℃下融化并存放，可能稳定达数月。

2）如果没有特殊原因而避免使用叠氮钠，亦可加入叠氮钠，浓度为0．02％。将纯化的抗体样本分装成合适的体积，于-20℃保存。

3）纯化的抗体溶液应以较高的浓度(如lmg／m1)在中性pH下保存。：常用的抗体储存浓度高达l0mg/ml。较低浓度的抗体冻存前应浓缩。所有标准的浓缩方法(如超滤法)，皆可使用。还有一个简单的方法是用蛋白A或蛋白G亲和柱来浓缩溶液。如果纯化的抗体不是用于标记，可将它们以较低浓度储存于加有1％BSA的溶液中。

4）经纯化制备的抗体在常用的缓冲液中是稳定的。其DH应保持在中性左右。如果pH在7-8之间，即使保存多年，对抗体也无损害。多数情况下，盐浓度适于保持在0-150mmol/L之间，但在长期存放的抗体中，盐溶液浓度高达500mmol/L时，对间隙连接蛋白47抗体能有损害。如果没有其他说明．律议用PBS或50mmol/LTris(DH8．0)溶液长期存放抗体。  

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

产品类型  一抗    

研究领域  神经生物学 信号转导 细胞骨架 细胞外基质  

蛋白分子量  predicted molecular weight: 47kDa 

性    状  Lyophilized or Liquid 

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  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 

产品应用   WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  Flow-Cyt=1:100-500  ICC=1:100-500  IF=1:100-500 

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

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

间隙连接蛋白47抗体产品介绍 

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 : UniProtKB/Swiss-Prot: Q5T442.1