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顶部产品介绍抗原信息化学信息储存和溶解信息计算器

Anti-NHLRC1 Polyclonal Antibody

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货号 TMAB-09469

Anti-NHLRC1 Polyclonal Antibody 是一种 Rabbit 抗体，靶向 NHLRC1。Anti-NHLRC1 Polyclonal Antibody 可用于 WB。

Anti-NHLRC1 Polyclonal Antibody

Anti-NHLRC1 Polyclonal Antibody

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Rating icon 还可以
货号 TMAB-09469

Anti-NHLRC1 Polyclonal Antibody 是一种 Rabbit 抗体，靶向 NHLRC1。Anti-NHLRC1 Polyclonal Antibody 可用于 WB。

规格价格库存数量
50 μL
¥ 1,165
5日内发货
100 μL
¥ 1,965
5日内发货
200 μL
¥ 2,780
5日内发货
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产品介绍

生物活性
产品描述
Anti-NHLRC1 Polyclonal Antibody is a Rabbit antibody targeting NHLRC1. Anti-NHLRC1 Polyclonal Antibody can be used in WB.
Ig Type
IgG
反应种属
Rat (predicted:Human,Mouse,Rabbit)
应用WB
推荐剂量
WB: 1:500-2000
抗体种类
Polyclonal
宿主来源Rabbit
亚细胞定位Endoplasmic reticulum. Nucleus. Localizes at the endoplasmic reticulum and, to a lesser extent, in the nucleus.
组织特异性Expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas.
构建方式Polyclonal Antibody
纯化方式Protein A purified
性状Liquid
缓冲液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
浓度1mg/ml
研究背景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.
抗原信息
免疫原
KLH conjugated synthetic peptide: human NHLRC1
抗原种属
Human
基因名称
NHLRC1
基因ID
378884
蛋白名称
E3 ubiquitin-protein ligase NHLRC1
Uniprot ID
Q3SYB1
功能
E3 ubiquitin-protein ligase which in complex with EPM2A/laforin and HSP70 suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Ubiquitinates PPP1R3C/PTG in a laforin-dependent manner, and targets it for proteasome-dependent degradation and this degradation decreases glycogen accumulation. Polyubiquitinates EPM2A/laforin and ubiquitinates AGL and targets them for proteasome-dependent degradation.
化学信息
分子量Theoretical: 42 kDa.
储存&溶解度
储存方式Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles.
运输方式Shipping with blue ice.
存储store at low temperature | -20°C for 1 year

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