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Invitrogen
NHLRC1 Polyclonal Antibody
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NHLRC1 Antibody (PA5-121441) in IHC (P)
Immunohistochemistry (Paraffin) analysis of NHLRC1 using NHLRC1 Polyclonal Antibody (Product # PA5-121441) at a dilution of 1:140 in human brain tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30 min at RT. Then primary antibody (1% BSA) was incubated at 4°C o... View More
Please note: We are reviewing Western blot images included in the antibody testing data in our catalog, including those provided by third parties. Unless expressly labeled or annotated as “raw-unedited”, Western blot images included in the antibody testing data in our catalog may have been edited, optimized or otherwise adjusted for presentation.
产品信息
PA5-121441
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Recombinant Human E3 ubiquitin-protein ligase NHLRC1 protein (86-395aa)
偶联物
Unconjugated
Unconjugated
Unconjugated
形式
Liquid
浓度
3.63 mg/mL
规格
363 µg
纯化类型
Protein G
保存液
PBS, pH 7.4, with 50% glycerol
内含物
0.03% ProClin 300
保存条件
-20°C or -80°C if preferred
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Antibody tested in Indirect ELISA
靶标信息
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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生物信息学
蛋白别名: E3 ubiquitin-protein ligase NHLRC1; Malin; NHL repeat-containing protein 1; RING-type E3 ubiquitin transferase NHLRC1
基因别名: EPM2B; NHLRC1
Entrez Gene ID: (Human) 378884
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