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NHLRC1 抗体

NHLRC1 适用: 人, 大鼠, 小鼠 WB, IF (p), IHC (p) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1387027
发货至: 中国
  • 抗原 See all NHLRC1 抗体
    NHLRC1 (NHL Repeat Containing 1 (NHLRC1))
    适用
    • 18
    • 3
    • 1
    • 1
    人, 大鼠, 小鼠
    宿主
    • 16
    • 3
    克隆类型
    • 16
    • 3
    多克隆
    标记
    • 10
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This NHLRC1 antibody is un-conjugated
    应用范围
    • 9
    • 8
    • 5
    • 2
    • 1
    • 1
    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
  • 应用备注
    WB 1:300-5000
    IHC-P 1:200-400
    IF(IHC-P) 1:50-200
    限制
    仅限研究用
  • 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
  • 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

    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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