Anti-SPG7抗体,痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体产品详细资料

产品编号 BYK-11761R

英文名称 SPG7
中文名称 痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体
别    名 CAR; Paraplegin; Cell adhesion regulator; Cell matrix adhesion regulator; CMAR; Paraplegin; PGN; Spastic paraplegia 7 (pure and complicated autosomal recessive); Spastic paraplegia 7 homolog (human); Spastic paraplegia 7, paraplegin (pure and complicated autosomal recessive); Spastic paraplegia protein 7; SPG5C; SPG7 gene; SPG7_HUMAN.
研究领域 心血管  细胞生物  神经生物学  细胞粘附分子  
分 子 量 88kDa
细胞定位 细胞浆 细胞膜 

抗体来源 Mouse or Rabbit
克隆类型 Monoclonal or Polyclonal
产品应用 WB、ELISA、IHC-P、IHC-F、Flow-Cyt、IF、IP、ICC 此产品应用不做依据，具体产品应用与实验稀释比请！

not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

性    状 Lyophilized or Liquid
浓    度 1mg/1ml
亚    型 IgG
纯化方法 affinity purified by Protein G
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20℃ 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℃. 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℃.


     Anti-SPG7抗体,痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体产品介绍 

      Paraplegin is a 795 amino acid metalloprotease that is a member of the AAA protein family. Localized to the mitochrondrial membrane and expressed throughout the body, Paraplegin is a multi-pass membrane protein that is thought to be involved in signal transduction and chaperone-like activities in the mitochrondria. Defects in the gene encoding Paraplegin are the cause of spastic paraplegia type 7 (SPG7), a form of autosomal recessive hereditary spastic paraplegia (AR-HSP). HSPs are degenerative spinal cord disorders that are characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence. Recent studies suggest that SPG7 may be a mitochondrial-based disease, as mutations in the Paraplegin gene lead to ragged-red fibers, oxidase-negative fibers and intense succinate dehydrogenase-stained areas of the mitochrondria. These mitochondrial dysfunctions lead to axonal degeneration and impaired axonal transport, thus causing the neurodegeneration seen in HSPs.

 此相关标记有：Alexa Fluor 350 标记、Alexa Fluor 488 标记、Alexa Fluor 555 标记、Alexa Fluor 647 标记、AP标记、APC标记、Biotin标记、Cy3标记、Cy5标记、Cy5.5标记、Cy7标记、FITC标记、Gold标记、HRP标记、PE标记、PE-Cy3标记、PE-CY5标记、PE-CY5.5标记、PE-CY7标记、RBITC标记 

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