• 靶点：
• MVK

• 简介：
• >>Terpenoid backbone biosynthesis;>>Metabolic pathways;>>Peroxisome

• 基因名称：
• MVK

• 蛋白名称：
• Mevalonate kinase

• Human Gene Id：
• 4598

• Human Swiss Prot No：
• Q03426

• Mouse Swiss Prot No：
• Q9R008

• 免疫原：
• The antiserum was produced against synthesized peptide derived from human Mevalonate Kinase. AA range:151-200

• 特异性：
• MVK Polyclonal Antibody detects endogenous levels of MVK protein.

• 组成：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• WB 1:500 - 1:2000. IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:10000. Not yet tested in other applications.

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 其他名称：
• MVK;Mevalonate kinase;MK

• 实测条带：
• 42kD

• 背景：
• This gene encodes the peroxisomal enzyme mevalonate kinase. Mevalonate is a key intermediate, and mevalonate kinase a key early enzyme, in isoprenoid and sterol synthesis. Mevalonate kinase deficiency caused by mutation of this gene results in mevalonic aciduria, a disease characterized psychomotor retardation, failure to thrive, hepatosplenomegaly, anemia and recurrent febrile crises. Defects in this gene also cause hyperimmunoglobulinaemia D and periodic fever syndrome, a disorder characterized by recurrent episodes of fever associated with lymphadenopathy, arthralgia, gastrointestinal dismay and skin rash. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2014],

• 功能：
• catalytic activity:ATP + (R)-mevalonate = ADP + (R)-5-phosphomevalonate.,disease:Defects in MVK are the cause of hyperimmunoglobulinemia D and periodic fever syndrome (HIDS) [MIM:260920]. HIDS is an autosomal recessive disease characterized by recurrent episodes of unexplained high fever associated with skin rash, diarrhea, adenopathy (swollen, tender lymph nodes), athralgias and/or arthritis. Concentration of IgD, and often IgA, are above normal.,disease:Defects in MVK are the cause of mevalonic aciduria [MIM:610377]. It is an accumulation of mevalonic acid which causes a variety of symptoms such as psychomotor retardation, dysmorphic features, cataracts, hepatosplenomegaly, lymphadenopathy, anemia, hypotonia, myopathy, and ataxia.,enzyme regulation:Farnesyl- and geranyl-pyrophosphates are competitive inhibitors.,function:May be a regulatory site in cholesterol biosynthetic pathway.,onl

• 细胞定位：
• Cytoplasm . Peroxisome .

• 组织表达：
• Brain,Hepatoma,Skin,Testis,

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