产品说明
一般描述
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. (provided by RefSeq)
免疫原
MVK (AAH16140, 297 a.a. ~ 396 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
LIDMNQHHLNALGVGHASLDQLCQVTRARGLHSKLTGAGGGGCGITLLKPGLEQPEVEATKQALTSCGFDCLETSIGAPGVSIHSATSLDSRVQQALDGL
外形
Solution in phosphate buffered saline, pH 7.4
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
NACRES | NA.41 |
产品性质
生物来源 | mouse |
偶联物 | unconjugated |
抗体形式 | purified immunoglobulin |
antibody product type | primary antibodies |
克隆 | 2C5, monoclonal |
形式 | buffered aqueous solution |
分子量 | antigen ~34.11 kDa |
species reactivity | human |
technique(s) | capture ELISA: suitable immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable indirect ELISA: suitable western blot: 1-5 μg/mL |
同位素/亚型 | IgG2aκ |
NCBI登记号 | BC016140 |
UniProt登记号 | Q03426 |
运输 | dry ice |
储存温度 | −20℃ |
Gene Information | human ... MVK(4598) |
安全信息
储存分类代码 | 13 - Non Combustible Solids |
WGK | WGK 1 |
闪点(F) | Not applicable |
闪点(C) | Not applicable |
Sigma-Aldrich