| 產(chǎn)品編號(hào) | bs-20155R |
| 英文名稱 | Rabbit Anti-MVK antibody |
| 中文名稱 | 甲羥戊酸激酶抗體 |
| 別 名 | LH receptor mRNA binding protein; LRBP; Mevalonate kinase; Mevalonic aciduria; MK antibody MVLK; KIME_HUMAN. |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 轉(zhuǎn)錄調(diào)節(jié)因子 激酶和磷酸酶 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 42kDa |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MVK: 101-200/396 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | Preservative: 0.02% Proclin300, Constituents: 1% BSA, 0.01M PBS, pH7.4. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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 |
