Anti-MVK Antibody Picoband® (ABIN7599941). Tested in WB, IHC, ICC/IF, Flow Cytometry, ELISA applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
纯化方法
Immunogen affinity purified.
免疫原
E.coli-derived human MVK recombinant protein (Position: K13-D394). Human MVK shares 80.8% and 81.4% amino acid (aa) sequence identity with mouse and rat MVK, respectively.
Western blot, 0.25-0.5 μg/mL, Human Immunohistochemistry, 2-5 μg/mL, Human Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human ELISA, 0.1-0.5 μg/mL, - 1. Balgobind, B., Wittebol-Post, D., Frenkel, J. Retinitis pigmentosa in mevalonate kinase deficiency. J. Inherit. Metab. Dis. 28: 1143-1145, 2005. 2. Brito, T., Banganho, D., Pedrosa, C., Neves, J. F. Homozygous V377I mutation causing mevalonate kinase. BMJ Case Rep. 15: e249135, 2022. 3. Cuisset, L., Drenth, J. P. H., Simon, A., Vincent, M. F., van der Velde Visser, S., van der Meer, J. W. M., Grateau, G., Delpech, M., International Hyper-IgD Study Group. Molecular analysis of MVK mutations and enzymatic activity in hyper-IgD and periodic fever syndrome. Europ. J. Hum. Genet. 9: 260-266, 2001.
限制
仅限研究用
状态
Lyophilized
溶解方式
Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.
抗原
MVK
(Mevalonate Kinase (MVK))
别名
MVK
背景
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.