NHLRC1 抗体
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- 抗原 See all NHLRC1 抗体
- NHLRC1 (NHL Repeat Containing 1 (NHLRC1))
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适用
- 人, 大鼠, 小鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This NHLRC1 antibody is un-conjugated
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应用范围
- Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
- 交叉反应
- 人, 小鼠, 大鼠
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human NHLRC1
- 亚型
- IgG
- Top Product
- Discover our top product NHLRC1 Primary Antibody
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- 应用备注
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WB 1:300-5000
IHC-P 1:200-400
IF(IHC-P) 1:50-200 - 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- 4 °C,-20 °C
- 储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
- 有效期
- 12 months
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- 抗原
- NHLRC1 (NHL Repeat Containing 1 (NHLRC1))
- 别名
- NHLRC1 (NHLRC1 产品)
- 别名
- EPM2A antibody, EPM2B antibody, MALIN antibody, bA204B7.2 antibody, AI505271 antibody, B230309E09Rik antibody, Epm2b antibody, malin-like antibody, NHL repeat containing E3 ubiquitin protein ligase 1 antibody, NHL repeat containing 1 antibody, NHLRC1 antibody, Nhlrc1 antibody
- 背景
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Synonyms: E3 ubiquitin-protein ligase NHLRC1, EPM2A, EPM2B, Malin, MGC119262, MGC119264, NHL repeat containing 1, NHL repeat containing protein 1, NHL repeat-containing protein 1, NHLC1_HUMAN, NHLRC 1, Nhlrc1.
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, 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.
- 基因ID
- 378884
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