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產品詳情
  • 產品名稱:Anti-SPG7抗體

  • 產品型號:痙攣性截癱蛋白7/基質細胞粘附調節(jié)蛋白抗體
  • 產品廠商:KALANG
  • 產品文檔:
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簡單介紹:
Anti-SPG7抗體產品質量穩(wěn)定,實驗效果明顯,貨期快,價格優(yōu)惠,歡迎垂詢訂購!我公司長期供應**組化抗體、WB抗體、**組化試劑盒和抗體試驗所需全部相關試劑、熒光標記抗體、單克隆抗體、多克隆抗體、各種標記的二抗IgG/IgM/IgD/IgA等科研實驗抗體。Anti-SPG7抗體用于**組化實驗,WB實驗,相應的標記抗體有HRP標記抗體,FITC標記,BIO等。
詳情介紹:
Rabbit  Anti-SPG7
Cat. Number:
Anti-SPG7抗體KL-11761R
Quantity size:
0.2ml
Concentration:
1mg/ml   Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
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 Anti-SPG7抗體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.
Also known as:
CAR; Paraplegin; Cell adhesion Anti-SPG7抗體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.
Specificity:
Rabbit Polyclonal IgG, affinity purified by Protein A.
Reacts with: Human, Mouse, Rat, .
Immunogen: KLH conjugated Anti-SPG7抗體synthetic peptide derived from human Paraplegin.
Predicted Molecular Weight: 88kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
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   
Not yet tested in other applications. Anti-SPG7抗體
Optimal working dilutions must be determined by the end user.

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