间隙连接蛋白47抗体
规格：1尘驳/1尘濒
英文名： GJC2
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 g
分子量： 47kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human GJC2/Con
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, Sheep, Guinea Pig,
细胞定位：细胞膜
间隙连接蛋白47抗体产物介绍：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 间隙连接蛋白47抗体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,间隙连接蛋白47抗体 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. Gene ID: 57165 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.
间隙连接蛋白47抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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.
研究领域：神经生物学 信号转导 细胞骨架 细胞外基质
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid