Full Product Name
Anti-MVK Antibody
Product Gene Name
anti-MVK antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Specificity
Western blot analysis of extracts of various cell lines, using MVK antibody.
Purity/Purification
Affinity Purification
Concentration
1mg/ml (lot specific)
Immunogen
Recombinant protein of human MVK
Storage Buffer
PBS with 0.02% sodium azide,50% glycerol, pH7.3.
Preparation and Storage
Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.
Other Notes
Small volumes of anti-MVK antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-MVK antibody
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. Two transcript variants that encode the same protein have been found for this gene.
Applications Tested/Suitable for anti-MVK antibody
Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-MVK antibody
WB: 1:500 - 1:2000
IHC: 1:50- 1:200
Western Blot (WB) of anti-MVK antibody
NCBI/Uniprot data below describe general gene information for MVK. It may not necessarily be applicable to this product.
NCBI Accession #
AKI69730.1
[Other Products]
UniProt Related Accession #
Q03426[Other Products]
Molecular Weight
42,451 Da
NCBI Official Full Name
MVK, partial
NCBI Official Synonym Full Names
mevalonate kinase
NCBI Official Symbol
MVK??[Similar Products]
NCBI Official Synonym Symbols
MK; LRBP; MVLK; POROK3
??[Similar Products]
NCBI Protein Information
mevalonate kinase
UniProt Protein Name
Mevalonate kinase
Protein Family
Mevalonate kinase
UniProt Gene Name
MVK??[Similar Products]
UniProt Synonym Gene Names
MK??[Similar Products]
NCBI Summary for MVK
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]
UniProt Comments for MVK
MVK: May be a regulatory site in cholesterol biosynthetic pathway. Defects in MVK are the cause of mevalonic aciduria (MEVA). 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. Defects in MVK are the cause of hyperimmunoglobulinemia D and periodic fever syndrome (HIDS). 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. Belongs to the GHMP kinase family. Mevalonate kinase subfamily.
Protein type: EC 2.7.1.36; Kinase, other; RNA-binding; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Translation
Chromosomal Location of Human Ortholog: 12q24.11
Cellular Component: cytosol; peroxisome
Molecular Function: identical protein binding; mevalonate kinase activity; protein binding
Biological Process: cholesterol biosynthetic process; isopentenyl diphosphate biosynthetic process, mevalonate pathway; isoprenoid biosynthetic process; negative regulation of inflammatory response
Disease: Hyper-igd Syndrome; Mevalonic Aciduria; Porokeratosis 3, Disseminated Superficial Actinic Type
Research Articles on MVK
1. this study extends the mutation spectrum of MVK; MVK protein stability and correct folding might be the molecular mechanism causing disseminated superficial actinic porokeratosis
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
