Rabbit anti-Human DGUOK Polyclonal Antibody | anti-DGUOK antibody
DGUOK, NT (Deoxyguanosine Kinase, Mitochondrial, dGK) (PE)
FLISA: 1:1,000
Applications are based on unconjugated antibody.
Western Blot (WB)
(Western blot analysis of anti-hDGUOK-M1 Pab in 293 cell line lysate (35ug/lane). hDGUOK-M1(arrow) was detected using the purified Pab.)
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
DGUOK: Required for the phosphorylation of several deoxyribonucleosides and certain nucleoside analogs widely employed as antiviral and chemotherapeutic agents. Defects in DGUOK are the cause of mitochondrial DNA depletion syndrome type 3 (MTDPS3). A disorder 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. Belongs to the DCK/DGK family. 5 isoforms of the human protein are produced by alternative splicing.
Protein type: Kinase, other; Nucleotide Metabolism - purine; EC 2.7.1.113; Mitochondrial
Chromosomal Location of Human Ortholog: 2p13
Cellular Component: mitochondrion; mitochondrial matrix; cytosol; nucleus
Molecular Function: nucleoside kinase activity; deoxyguanosine kinase activity; ATP binding
Biological Process: purine deoxyribonucleoside metabolic process; nucleotide biosynthetic process; nucleobase, nucleoside and nucleotide metabolic process; dGTP metabolic process; guanosine metabolic process; protein amino acid phosphorylation; purine base metabolic process; purine salvage; deoxyribonucleoside monophosphate biosynthetic process
Disease: Mitochondrial Dna Depletion Syndrome 3 (hepatocerebral Type)