Product datasheet Anti-MVK antibody [AT14F6] ab177525 1 Image Overview Product name Anti-MVK antibody [AT14F6] Description Mouse monoclonal [AT14F6] to MVK Tested applications ELISA, WB Species reactivity Reacts with: Human Immunogen Recombinant full length protein corresponding to Human MVK aa 1-396. Purified from E. coli. (NP_001107657) Sequence: MLSEVLLVSAPGKVILHGEHAVVHGKVALAVSLNLRTFLRLQPHSNGKVD LSLPNIGIKRAWDVARLQSLDTSFLEQGDVTTPTSEQVEKLKEVAGLPDD CAVTERLAVLAFLYLYLSICRKQRALPSLDIVVWSELPPGAGLGSSAAYS VCLAAALLTVCEEIPNPLKDGDCVNRWTKEDLELINKWAFQGERMIHGNP SGVDNAVSTWGGALRYHQGKISSLKRSPALQILLTNTKVPRNTRALVAGV RNRLLKFPEIVAPLLTSIDAISLECERVLGEMGEAPAPEQYLVLEELIDM NQHHLNALGVGHASLDQLCQVTRARGLHSKLTGAGGGGCGITLLKPGL EQPEVEATKQALTSCGFDCLETSIGAPGVSIHSATSLDSRVQQALDGL Database link: Q03426 Run BLAST with Run BLAST with Positive control HepG2 cell line lysate General notes Anti-Human MVK mAb, clone AT14F6, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant Human MVK protein. Properties Form Liquid Storage instructions Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. Purity Protein A purified Clonality Monoclonal Clone number AT14F6 Isotype IgG3 Light chain type kappa 1 Applications Our Abpromise guarantee covers the use of ab177525 in the following tested applications. The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user. Application Abreviews Notes ELISA Use at an assay dependent concentration. WB 1/500 - 1/5000. Predicted molecular weight: 42 kDa. Target Function May be a regulatory site in cholesterol biosynthetic pathway. Pathway Isoprenoid biosynthesis; isopentenyl diphosphate biosynthesis via mevalonate pathway; isopentenyl diphosphate from (R)-mevalonate: step 1/3. Involvement in disease Defects in MVK are the cause of mevalonic aciduria (MEVA) [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. 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. Sequence similarities Belongs to the GHMP kinase family. Mevalonate kinase subfamily. Cellular localization Cytoplasm. Peroxisome. Anti-MVK antibody [AT14F6] images Lane 1 : Anti-MVK antibody [AT14F6] (ab177525) at 1/500 dilution Lane 2 : Anti-MVK antibody [AT14F6] (ab177525) at 1/1000 dilution Lane 3 : Anti-MVK antibody [AT14F6] (ab177525) at 1/5000 dilution Lane 1 : HepG2 cell line lysate Lane 2 : HepG2 cell line lysate Lane 3 : HepG2 cell line lysate Western blot - Anti-MVK [AT14F6] antibody Lysates/proteins at 40 µg per lane. (ab177525) Secondary Goat anti-mouse HRP conjugated antibody developed using the ECL technique Predicted band size : 42 kDa 2 Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE" Our Abpromise to you: Quality guaranteed and expert technical support Replacement or refund for products not performing as stated on the datasheet Valid for 12 months from date of delivery Response to your inquiry within 24 hours We provide support in Chinese, English, French, German, Japanese and Spanish Extensive multi-media technical resources to help you We investigate all quality concerns to ensure our products perform to the highest standards If the product does not perform as described on this datasheet, we will offer a refund or replacement. For full details of the Abpromise, please visit http://www.abcam.com/abpromise or contact our technical team. Terms and conditions Guarantee only valid for products bought direct from Abcam or one of our authorized distributors 3