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货号： bs-20155R 基本售价： 1380.0 元 规格： 100ul
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-20155R
- 英文名称
- MVK
- 中文名称
- 甲羟戊酸激酶抗体
- 别 名
- LH receptor mRNA binding protein; LRBP; Mevalonate kinase; Mevalonic aciduria; MK antibody MVLK; KIME_HUMAN.
- 规格价格
- 100ul/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 100ul 200ul
- 研究领域
- 肿瘤 细胞生物 免疫学 转录调节因子 激酶和磷酸酶
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human,
- 产品应用
- ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 42kDa
- 细胞定位
- 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human MVK:101-200/396
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- PubMed
- PubMed
- 产品介绍
- background:
MVK 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 MVK results in mevalonic aciduria.
Function:
May be a regulatory site in cholesterol biosynthetic pathway.
Subunit:
Homodimer.
Subcellular Location:
Cytoplasm.
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.
Similarity:
Belongs to the GHMP kinase family. Mevalonate kinase subfamily.
SWISS:
Q03426
Gene ID:
4598
Database links:Entrez Gene: 4598 Human
Omim: 251170 Human
SwissProt: Q03426 Human
Unigene: 130607 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.