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Rabbit Anti-Thymidine kinase 2/BF350 Conjugated antibody (bs-19665R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-19665R-BF350
英文名稱 Rabbit Anti-Thymidine kinase 2/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的胸苷激酶2抗體
別    名 EC 2.7.1.21; KITM_HUMAN; mitochondrial; Mt TK; Mt-TK; Thymidine kinase 2; Thymidine kinase 2 mitochondrial; TK2.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  線粒體  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 31kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Thymidine kinase 2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)品介紹 background:
This gene encodes a deoxyribonucleoside kinase that specifically phosphorylates thymidine, deoxycytidine, and deoxyuridine. The encoded enzyme localizes to the mitochondria and is required for mitochondrial DNA synthesis. Mutations in this gene are associated with a myopathic form of mitochondrial DNA depletion syndrome. Alternate splicing results in multiple transcript variants encoding distinct isoforms, some of which lack transit peptide, so are not localized to mitochondria. [provided by RefSeq, Dec 2012].

Function:
Deoxyribonucleoside kinase that phosphorylates thymidine, deoxycytidine, and deoxyuridine. Also phosphorylates anti-viral and anti-cancer nucleoside analogs.

Subcellular Location:
Mitochondrion.

Tissue Specificity:
Predominantly expressed in liver, pancreas, muscle, and brain.

DISEASE:
Defects in TK2 are a cause of mitochondrial DNA depletion syndrome type 2 (MTDPS2) [MIM:609560]. A disorder characterized primarily by childhood onset of muscle weakness associated with depletion of mtDNA in skeletal muscle. There is wide clinical variability; some patients have onset in infancy and show a rapidly progressive course with early death due to respiratory failure, whereas others have later onset of a slowly progressive myopathy.

Similarity:
Belongs to the DCK/DGK family.

Database links:

Entrez Gene: 7084 Human

Entrez Gene: 57813 Mouse

Omim: 188250 Human

SwissProt: O00142 Human

SwissProt: Q9R088 Mouse

Unigene: 512619 Human

Unigene: 183110 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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