Rabbit anti-Human MVK Polyclonal Antibody | anti-MVK antibody

MVK, NT (Mevalonate Kinase, MK, KIME) (Azide free) (HRP)

Cat. Num. AAA6488713

• Gene Names: MVK; MK; LRBP; MVLK; POROK3
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Purified by Protein G Affinity Chromatography.
• Synonyms: MVK; Polyclonal Antibody; NT (Mevalonate Kinase; MK; KIME) (Azide free) (HRP); anti-MVK antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human MVK.
• Purity/Purification: Purified by Protein G Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish Peroxidase (HRP).

• Applicable Applications for anti-MVK antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-1:500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the N-terminal region of human MVK (KLH).
• Conjugate: HRP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of MVP (arrow) using rabbit polyclonal MVP Antibody (N-term). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the MVK gene (Lane 2).)

Product Categories/Family for anti-MVK antibody

Antibodies; Enzymes

References

1. Stojanov, S., et al., Arthritis Rheum. 50(6):1951-1958 (2004). 2. Nair, A.K., et al., J. Biol. Chem. 279(15):14937-14944 (2004). 3. Simon, A., et al., Neurology 62(6):994-997 (2004). 4. Houten, S.M., et al., J. Biol. Chem. 278(8):5736-5743 (2003). 5. Houten, S.M., et al., Eur. J. Hum. Genet. 11(2):196-200 (2003).

NCBI and Uniprot Product Information

• NCBI GI #: 4557769
• NCBI GeneID: 4598
• NCBI Accession #: NP_000422
• NCBI GenBank Nucleotide #: NM_000431
• UniProt Accession #: Q03426
• Molecular Weight: 42,451 Da
• NCBI Official Full Name: mevalonate kinase isoform a
• NCBI Official Synonym Full Names: mevalonate kinase
• NCBI Official Symbol: MVK
• NCBI Official Synonym Symbols: MK; LRBP; MVLK; POROK3
• NCBI Protein Information: mevalonate kinase; LH receptor mRNA-binding protein; mevalonate kinase 1
• UniProt Protein Name: Mevalonate kinase
• Protein Family: Mevalonate kinase
• UniProt Gene Name: MVK
• UniProt Synonym Gene Names: MK
• UniProt Entry Name: KIME_HUMAN

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; Translation; RNA-binding; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis

Chromosomal Location of Human Ortholog: 12q24

Cellular Component: peroxisome; cytosol

Molecular Function: identical protein binding; mevalonate kinase activity; ATP binding

Biological Process: isoprenoid biosynthetic process; isopentenyl diphosphate biosynthetic process, mevalonate pathway; negative regulation of inflammatory response; phosphorylation; cholesterol biosynthetic process

Disease: Porokeratosis 3, Disseminated Superficial Actinic Type; Hyper-igd Syndrome; Mevalonic Aciduria