NHLRC1 抗体
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北京 101111
Quick Overview for NHLRC1 抗体 (ABIN1387027)
抗原
See all NHLRC1 抗体适用
宿主
克隆类型
标记
应用范围
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交叉反应
- 人, 小鼠, 大鼠
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human NHLRC1
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亚型
- IgG
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anti-NHL Repeat Containing 1 (NHLRC1) (AA 1-280) antibody
NHLRC1 适用: 人 WB, ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-NHL Repeat Containing 1 (NHLRC1) (AA 137-246) antibodyNHLRC1 适用: 人 WB, ELISA 宿主: 小鼠 Monoclonal 3G6 unconjugated
anti-NHL Repeat Containing 1 (NHLRC1) (AA 86-395) antibodyNHLRC1 适用: 人 ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-NHL Repeat Containing 1 (NHLRC1) (AA 1-280) antibodyNHLRC1 适用: 人 WB, ELISA 宿主: 兔 Polyclonal unconjugated
anti-NHL Repeat Containing 1 (NHLRC1) antibodyNHLRC1 适用: 人 WB, IF, IC 宿主: 兔 Polyclonal unconjugated
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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
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浓度
- 1 μg/μL
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缓冲液
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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有效期
- 12 months
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- NHLRC1 (NHL Repeat Containing 1 (NHLRC1))
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别名
- NHLRC1
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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.
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基因ID
- 378884
抗原
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