外周髓鞘蛋白-22重组兔单克隆抗体

别    名 GAS3; CMT1A; CMT1E; DSS; GAS-3; Growth Arrest Specific 3; Growth arrest-specific protein 3; HMSNIA; HNPP; MGC20769; Peripheral Myelin Protein 22; PMP-22; PMP22; PMP22_HUMAN; Sp110; Trembler.   

研究领域 免疫学  神经生物学  糖蛋白   

抗体来源 Rabbit 

克隆类型 Monoclonal 

克 隆 号 1A11 

交叉反应 (predicted: Human, Rat, ) 

产品应用 IHC-P=1:200-400 ICC=1:50-200 IF=1:50-200 （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. 

分 子 量 22kDa 

细胞定位 细胞膜  

性    状 Lyophilized or Liquid 

浓    度 1mg/ml 

免 疫 原 Full length protein:  

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. 

PubMed PubMed 

产品介绍 PMP22 is a 22 kDa glycoprotein expressed in the compact myelin of the peripheral nervous system. In the peripheral nervous system, PMP 22 is produced by myelinating Schwann cells and is coexpressed with the genes for myelin basic protein (MBP) during nerve development and regeneration. Alterations in the level of this protein cause several genetic human diseases. If the protein is duplicated, patients develop Charcot Marie Tooth disease. If one copy of the gene is deleted, they suffer from the inherited tendency to pressure palsies.

 

Function:

Might be involved in growth regulation, and in myelinization in the peripheral nervous system.

 

Subcellular Location:

Cell membrane; Multi-pass membrane protein.

 

DISEASE:

Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1A (CMT1A) [MIM:118220]; also known as hereditary motor and sensory neuropathy IA. CMT1A is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Char

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