ITT2924
ITT2924
- Catalog: ITT2924
- Gene/Protein: MVK
- Product Description: Immunotag™ MVK Polyclonal Antibody
385.0000
Price in reward points: 385
Your shopping cart is empty!
Antibody Specification | |
Datasheet | |
Target Protein | MVK |
Clonality | Polyclonal |
Storage/Stability | -20°C/1 year |
Application | WB,IHC-p,IF,ELISA |
Recommended Dilution | Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/10000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human,Monkey |
Host Species | Rabbit |
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. |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Gene Name | MVK |
Accession No. | Q03426 Q9R008 |
Alternate Names | MVK; Mevalonate kinase; MK |
Description | mevalonate kinase(MVK) Homo sapiens 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], |
Cell Pathway/ Category | Terpenoid backbone biosynthesis, |
Protein Expression | Brain,Hepatoma,Skin,Testis, |
Subcellular Localization | cytoplasm,peroxisome,cytosol,extracellular exosome, |
Protein 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.,online information:Repertory of FMF and hereditary autoinflammatory disorders mutations,pathway:Isoprenoid biosynthesis; isopentenyl-PP biosynthesis via mevalonic acid pathway; isopentenyl-PP from (R)-mevalonic acid: step 1/3.,similarity:Belongs to the GHMP kinase family.,similarity:Belongs to the GHMP kinase family. Mevalonate kinase subfamily.,subunit:Homodimer., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |