產品詳情
簡單介紹：
Anti-Dysferlin interacting protein 1抗體產品質量穩定，實驗效果明顯，貨期快，價格優惠，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-Dysferlin interacting protein 1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-Dysferlin interacting protein 1
Cat. Number:
Anti-Dysferlin interacting protein 1抗體KL-14476R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Dysferlin is a muscle-specific protein that is essential for normal muscle function and development (1). Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.3-p13.1, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, *****-onset, distal dystrophy known as Miyoshi myopathy (MM) (1,2). Dysferlin localizes to the muscle fiber membrane, butAnti-Dysferlin interacting protein 1抗體 is absent in MM and LGMD-2B muscle (1,3). Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation (3). Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle (1). It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in *****hood (3). Identical mutations in the dysferlin gene can produce more than one myopathy phenotype, indicating that additional genes and/or other factors are also involved in the clinical phenotype (4,5). The DYSF gene has no homology to any other known mammalian gene, but the protein product is related to the spermatogenesis factor fer-1 of Caenorhabditis elegans. The name ‘dysferlin’ combines the role of the gene in producing muscular dystrophy with its homology to C. elegans (6).
Also known as:
dysferlin interacting protein 1 (toonin);Anti-Dysferlin interacting protein 1抗體 Dysfip1; PPR27_HUMAN; Toonin.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Dog, Cow, Horse, Sheep, .
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Immunogen: KLH conjugated synthetic peptide derived from human Dysferli.
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Predicted Molecular Weight: 17kDa.
Storage:
Shipped at 4℃, Store at -20℃ Anti-Dysferlin interacting protein 1抗體(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-Dysferlin interacting protein 1抗體
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications. Anti-Dysferlin interacting protein 1抗體
Optimal working dilutions must be determined by the end user.
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