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Rabbit Anti-MVK  antibody (bs-20155R)
~~~促销,代码KX240301~~~
~~~促销,代码KX240302~~~
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
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.  
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 (predicted: Human)
产品应用 IHC-P=1:100-500, IHC-F=1:100-500, ICC=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.
理论分子量 42kDa
细胞定位 细胞浆 
性    状 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
缓 冲 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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



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