| 產(chǎn)品編號(hào) | bs-14277R-BF488 |
| 英文名稱 | Rabbit Anti-DGUOK/BF488 Conjugated antibody |
| 中文名稱 | BF488標(biāo)記的脫氧鳥苷激酶抗體 |
| 別 名 | Deoxyguanosine kinase mitochondrial; dGK; Deoxyguanosine kinase; DGUOK; DGUOK_HUMAN; Deoxyguanosine kinase, mitochondrial. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書 | 100ul |
| 研究領(lǐng)域 | 免疫學(xué) 神經(jīng)生物學(xué) 激酶和磷酸酶 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep, ) |
| 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 | 28kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human DGUOK |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 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: In mammalian cells, the phosphorylation of purine deoxyribonucleosides is mediated predominantly by two deoxyribonucleoside kinases, cytosolic deoxycytidine kinase and mitochondrial deoxyguanosine kinase. The protein encoded by this gene is responsible for phosphorylation of purine deoxyribonucleosides in the mitochondrial matrix. In addition, this protein phosphorylates several purine deoxyribonucleoside analogs used in the treatment of lymphoproliferative disorders, and this phosphorylation is critical for the effectiveness of the analogs. Alternative splice variants encoding different protein isoforms have been described for this gene. [provided by RefSeq, Jul 2008] Function: Mitochondrial deoxyguanosine kinase (DGUOK) is required for the phosphorylation of several deoxyribonucleosides and certain purine deoxykribonucleoside analogs widely employed as antiviral and chemotherapeutic agents. Purine deoxyribonucleoside analogs are extensively used in treatment of lymphoproliferative disorders. These compounds are administered as pro-drugs, and their efficiency is dependent on intracellular phosphorylation to the corresponding triphosphates. In mammalian cells, the phosphorylation of purine deoxyribonucleosides is mediated predominantly by 2 deoxyribonucleoside kinases: cytosolic deoxycytidine kinase (DCK) and mitochondrial deoxyguanosine kinase (DGUOK also known as DGK). DGUOK expression is ubiquitous, with highest levels in muscle, brain, liver and lymphoid tissues. Defects in DGUOK are a cause of mitochondrial DNA depletion syndrome (MDS). MDS is a clinically heterogeneous group of disorders characterized by a reduction in mitochondrial DNA (mtDNA) copy number. Primary mtDNA depletion is inherited as an autosomal recessive trait and may affect single organs, typically muscle or liver, or multiple tissues. Mitochondrial DNA depletion syndromes are phenotypically heterogeneous, autosomal recessive disorders characterized by tissue-specific reduction in mtDNA copy number. Affected individuals with the hepatocerebral form of mtDNA depletion syndrome have early progressive liver failure and neurologic abnormalities, hypoglycemia, and increased lactate in body fluids. Subunit: Homodimer. Subcellular Location: Mitochondrion. Tissue Specificity: Ubiquitous. Highest expression in muscle, brain, liver and lymphoid tissues. DISEASE: Mitochondrial DNA depletion syndrome 3 (MTDPS3) [MIM:251880]: A disorder due to mitochondrial dysfunction characterized by onset in infancy of progressive liver failure, hypoglycemia, increased lactate in body fluids, and neurologic abnormalities including hypotonia, encephalopathy, peripheral neuropathy. Affected tissues show both decreased activity of the mtDNA-encoded respiratory chain complexes and mtDNA depletion. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the DCK/DGK family. Database links: Entrez Gene: 1716 Human Entrez Gene: 27369 Mouse Omim: 601465 Human SwissProt: Q16854 Human SwissProt: Q4ZG09 Human SwissProt: Q7L1W9 Human SwissProt: Q9BVK7 Human SwissProt: Q3TKB4 Mouse SwissProt: Q504N4 Mouse SwissProt: Q8CBU2 Mouse SwissProt: Q91XI5 Mouse SwissProt: Q9QX60 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
