Mouse MVK Monoclonal Antibody | anti-MVK antibody

Mouse monoclonal MVK antibody

Cat. Num. AAA5306809

• Gene Names: MVK; MK; LRBP; MVLK; POROK3
• Applications: Immunofluorescence, Western Blot
• Purity: Purified by affinity chromatography.
• Synonyms: MVK; Monoclonal Antibody; Mouse monoclonal MVK antibody; MVK antibody; anti-MVK antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG2a
• Clone Number: 1D7
• Purity/Purification: Purified by affinity chromatography.
• Form/Format: Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.
• Concentration: 500 ug-1 mg/ml (varies by lot)
• Sequence Length: 396

• Applicable Applications for anti-MVK antibody: Immunofluorescence (IF), Western Blot (WB)
• Application Notes: IF: 1:100; WB: 1:2000
• Immunogen: MVK antibody was raised in mouse using a full length recombinant protein of human MVK (NP_000422) produced in HEK293T cells, as the immunogen.
• Cross-Reactivity: Human
• Preparation and Storage: Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Immunofluorescence (IF)

(Immunofluorescent staining of COS7 cells transiently transfected with recombinant MVK protein using MVK antibody)

Product Categories/Family for anti-MVK antibody

Signal Transduction

NCBI and Uniprot Product Information

• NCBI GI #: 823669036
• NCBI GeneID: 4598
• Molecular Weight: 42,451 Da
• NCBI Official Full Name: MVK, partial
• NCBI Official Synonym Full Names: mevalonate kinase
• NCBI Official Symbol: MVK
• NCBI Official Synonym Symbols: MK; LRBP; MVLK; POROK3
• NCBI Protein Information: mevalonate kinase
• UniProt Protein Name: Mevalonate kinase
• Protein Family: Mevalonate kinase
• UniProt Gene Name: MVK
• UniProt Synonym Gene Names: MK

NCBI Description

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 Description

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