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Invitrogen
NHLRC1 Polyclonal Antibody
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NHLRC1 Antibody (PA5-121023) in IHC (P)
Immunohistochemistry analysis of NHLRC1 in paraffin-embedded human stomach. Samples were incubated with NHLRC1 Polyclonal antibody (Product # PA5-121023) using a dilution of 1:100 (40x lens). Perform microwave antigen retrieval with 10 mM PBS buffer pH 7.2 before commencing with IHC staining protocol.
产品信息
PA5-121023
应用
建议稀释比
已发表文章
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human NHLRC1 (NP_9409882)
偶联物
形式
Liquid
浓度
7.39 mg/mL
纯化类型
Affinity Chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.05% ProClin 300
保存条件
-20°C, Avoid Freeze/Thaw Cycles
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Positive test controls include: Mouse liver, Mouse brain, Mouse heart, Rat brain, Rat heart, Rat liver. The target is usually found in the following locations: Endoplasmic reticulum, Nucleus.
Immunogen sequence: MAAEASESGP ALHELMREAE ISLLECKVCF EKFGHRQQRR PRNLSCGHVV CLACVAALAH PRTLALECPF CRRACRGCDT SDCLPVLHLI ELLGSALRQS PAAHRAAPSA PGALTCHHTF GGWGTLVNPT GLALCPKTGR VVVVHDGRRR VKIFDSGGGC AHQFGEKGDA AQDIRYPVDV TITNDCHVVV TDAGDRSIKV FDFFGQIKLV IGGQFSLPWG VETTPQNGIV VTDAEAGSLH LLDVDFAEGV LRRTERLQAH LCNPRGVAVS WLTGAIAVLE
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: E3 ubiquitin-protein ligase NHLRC1; Malin; NHL repeat containing 1; NHL repeat-containing protein 1; RING-type E3 ubiquitin transferase NHLRC1
基因别名: AI505271; B230309E09Rik; bA204B7.2; EPM2A; EPM2B; MALIN; NHLRC1
UniProt ID: (Human) Q6IMH1, (Mouse) Q8BR37, (Rat) Q6IMG5
Entrez Gene ID: (Human) 378884, (Mouse) 105193, (Rat) 364682
ubiquitin-protein transferase activity
protein binding
zinc ion binding
ligase activity
ubiquitin protein ligase activity
UDP-N-acetylmuramoylalanyl-D-glutamyl-2,6-diaminopimelate-D-alanyl-D-alanine ligase activity
ribosomal S6-glutamic acid ligase activity
coenzyme F420-0 gamma-glutamyl ligase activity
coenzyme F420-2 alpha-glutamyl ligase activity
metal ion binding
protein-glycine ligase activity
protein-glycine ligase activity, initiating
protein-glycine ligase activity, elongating
tubulin-glycine ligase activity
protein-glutamic acid ligase activity
tubulin-glutamic acid ligase activity
ubiquitin-protein ligase
