Target：MVK

Fields：Terpenoid backbone biosynthesis;Metabolic pathways;Peroxisome

Gene Name：MVK

Protein Name：Mevalonate kinase

Human Gene Id：4598

Human Swiss Prot No：Q03426

Mouse Swiss Prot No：Q9R008

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

Specificity：MVK Polyclonal Antibody detects endogenous levels of MVK protein.

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

Source：Polyclonal, Rabbit,IgG

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

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

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

Other Name：MVK;Mevalonate kinase;MK

Observed Band(KD)：42kD

Background： 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],

Function：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

Subcellular Location：Cytoplasm . Peroxisome .

Expression： Brain,Hepatoma,Skin,Testis,