Anti-SPG7抗體,痙攣性截癱蛋白7/基質細胞粘附調節蛋白抗體說明產品詳細資料
產品編號 BYK-11761R
英文名稱 SPG7
中文名稱 痙攣性截癱蛋白7/基質細胞粘附調節蛋白抗體
別 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.
研究領域 心血管 細胞生物 神經生物學 細胞粘附分子
抗體來源 Mouse or Rabbit
克隆類型 Monoclonal or Polyclonal
產品應用 WB、ELISA、IHC-P、IHC-F、Flow-Cyt、IF、IP、ICC 此產品應用不做依據，具體產品應用與實驗稀釋比請！021-6034 0810
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
性 狀 Lyophilized or Liquid
濃 度 1mg/1ml
亞 型 IgG
純化方法 affinity purified by Protein G
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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抗體,痙攣性截癱蛋白7/基質細胞粘附調節蛋白抗體說明產品介紹：
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..
此相關標記有：Alexa Fluor 350 標記、Alexa Fluor 488 標記、Alexa Fluor 555 標記、Alexa Fluor 647 標記、AP標記、APC標記、Biotin標記、Cy3標記、Cy5標記、Cy5.5標記、Cy7標記、FITC標記、Gold標記、HRP標記、PE標記、PE-Cy3標記、PE-CY5標記、PE-CY5.5標記、PE-CY7標記、RBITC標記
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