產品詳情
簡單介紹：
Anti-NHLRC1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-NHLRC1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-NHLRC1
Cat. Number:
Anti-NHLRC1抗體KL-9331R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Progressive myoclonic epilepsy type 2 (EPM2), also called Lafora disease, is an autosomal recessive disease characterized by grand mal seizures and/or myoclonus at about 15 years of age. Rapid and severe mental deterioration follows, often with psychotic features. Survival is less than 10 years after onset. Starch-like, Anti-NHLRC1抗體endoplasmic reticulum-associated polyglucosans, called Lafora bodies, can be observed in brain, muscle, liver and heart. One cause of Lafora disease is due to mutations in NHLRC1, the gene encoding Malin. Forty-nine different mutations in NHLRC1 have been shown to cause EPM2. Malin, also called NHL repeat-containing protein 1, is a single subunit E3 ubiquitin ligase, containing 6 NHL repeats and 1 RING-type zinc finger. Malin’s RING domain is responsible for its ability to mediate ubiquitination. Malin interacts with and polyubiquitinates Laforin, a protein also implicated in EPM2. Malin localizes to the endoplasmic reticulum and, to a lesser extent, in the nucleus. Malin is expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas.
Also known as:
E3 ubiquitin-protein ligase NHLRC1;Anti-NHLRC1抗體 EPM2A; EPM2B; Malin; MGC119262; MGC119264; NHL repeat containing 1; NHL repeat containing protein 1; NHL repeat-containing protein 1; NHLC1_HUMAN; NHLRC 1; Nhlrc1.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Rabbit, .
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Immunogen: KLH conjugated syntheticAnti-NHLRC1抗體 peptide derived from human NHLRC1.
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Predicted Molecular Weight: 42kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-F=1:100-500 IF=1:50-200
Not yet tested in other applications. Anti-NHLRC1抗體
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications. Anti-NHLRC1抗體
Optimal working dilutions must be determined by the end user.
