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deoxyguanosine kinase, mitochondrial; dGK (DGUOK, DGK)
Function:
- required for the phosphorylation of several deoxyribonucleosides & certain nucleoside analogs widely employed as antiviral & chemotherapeutic agents
ATP + deoxyguanosine = ADP + dGMP
Structure:
- homodimer
- belongs to the DCK/DGK family
Compartment: mitochondria
Alternative splicing: named isoforms=5
Expression:
- ubiquitous
- highest expression in muscle, brain, liver & lymphoid tissues
Pathology:
- defects in DGUOK are the cause of mitochondrial DNA depletion syndrome type 3
General
kinase or ATP-phosphotransferase
Properties
SIZE: entity length = 277 aa
MW = 32 kD
COMPARTMENT: mitochondria
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 45-53
binding site
SITE: 70-70
FOR-BINDING-OF: Substrate
binding site
SITE: 100-100
FOR-BINDING-OF: Substrate
binding site
SITE: 111-111
FOR-BINDING-OF: Substrate
binding site
SITE: 118-118
FOR-BINDING-OF: Substrate
glutamate residue {E141}
binding site
SITE: 142-142
FOR-BINDING-OF: Substrate
binding site
SITE: 147-147
FOR-BINDING-OF: Substrate
ATP-binding site
NAME: ATP-binding site
SITE: 206-208
binding site
SITE: 211-211
FOR-BINDING-OF: Substrate
Database Correlations
OMIM correlations
MORBIDMAP 601465
UniProt Q16854
Pfam PF01712
Entrez Gene 1716
Kegg hsa:1716
ENZYME EC=2.7.1.113
References
- UniProt :accession Q16854
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/DGUOK