Anti-DGUOK, NT (Deoxyguanosine Kinase, Mitochondrial, dGK)

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. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: ELISA: 1:1,000 , Western Blot: 1:100-1:500, Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.