You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Identification
HMDB Protein ID HMDBP03815
Secondary Accession Numbers
  • 9403
Name Thymidine kinase 2, mitochondrial
Synonyms
  1. Mt-TK
Gene Name TK2
Protein Type Enzyme
Biological Properties
General Function Involved in ATP binding
Specific Function Deoxyribonucleoside kinase that phosphorylates thymidine, deoxycytidine, and deoxyuridine. Also phosphorylates anti-viral and anti-cancer nucleoside analogs.
Pathways
  • Drug metabolism - other enzymes
  • Pyrimidine metabolism
Reactions
Adenosine triphosphate + Thymidine → ADP + 5-Thymidylic acid details
Adenosine triphosphate + Deoxyuridine → ADP + dUMP details
Floxuridine + Adenosine triphosphate → 5-Fluorodeoxyuridine monophosphate + ADP details
GO Classification
Biological Process
deoxyribonucleotide metabolic process
mitochondrial DNA replication
pyrimidine nucleoside salvage
pyrimidine nucleobase metabolic process
Cellular Component
mitochondrial matrix
mitochondrial inner membrane
Function
binding
catalytic activity
transferase activity
transferase activity, transferring phosphorus-containing groups
nucleoside binding
purine nucleoside binding
adenyl nucleotide binding
adenyl ribonucleotide binding
atp binding
phosphotransferase activity, alcohol group as acceptor
Molecular Function
thymidine kinase activity
phosphotransferase activity, alcohol group as acceptor
ATP binding
Process
metabolic process
nitrogen compound metabolic process
cellular nitrogen compound metabolic process
nucleobase, nucleoside, nucleotide and nucleic acid metabolic process
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location 16
Locus 16q22-q23.1
SNPs TK2
Gene Sequence
>798 bp
ATGCTGCTGTGGCCGCTGCGGGGCTGGGCCGCCCGGGCGCTGCGCTGCTTTGGGCCGGGA
AGTCGCGGGAGCCCGGCCTCAGGCCCCGGGCCGCGGAGGGTGCAGCGCCGGGCCTGGCCT
CCCGATAAAGAACAGGAAAAAGAGAAAAAATCAGTGATCTGTGTCGAGGGCAATATTGCA
AGTGGGAAGACGACATGCCTGGAATTCTTCTCCAACGCGACAGACGTCGAGGTGTTAACG
GAGCCTGTGTCCAAGTGGAGAAATGTCCGTGGCCACAATCCTCTGGGCCTGATGTACCAC
GATGCCTCTCGCTGGGGTCTTACGCTACAGACTTATGTGCAGCTCACCATGCTGGACAGG
CATACTCGTCCTCAGGTGTCATCTGTACGGTTGATGGAGAGGTCGATTCACAGCGCAAGA
TACATTTTTGTAGAAAACCTGTATAGAAGTGGGAAGATGCCAGAAGTGGACTATGTAGTT
CTGTCGGAATGGTTTGACTGGATCTTGAGGAACATGGACGTGTCTGTTGATTTGATAGTT
TACCTTCGGACCAATCCTGAGACTTGTTACCAGAGGTTAAAGAAGAGATGCAGGGAAGAG
GAGAAGGTCATTCCGCTGGAATACCTGGAAGCAATTCACCATCTCCATGAGGAGTGGCTC
ATCAAAGGCAGCCTTTTCCCCATGGCAGCCCCTGTTCTGGTGATTGAGGCTGACCACCAC
ATGGAGAGGATGTTAGAACTCTTTGAACAAAATCGGGATCGAATATTAACTCCAGAGAAT
CGGAAGCATTGCCCATAG
Protein Properties
Number of Residues 265
Molecular Weight 27561.495
Theoretical pI 6.849
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Thymidine kinase 2, mitochondrial
MLLWPLRGWAARALRCFGPGSRGSPASGPGPRRVQRRAWPPDKEQEKEKKSVICVEGNIA
SGKTTCLEFFSNATDVEVLTEPVSKWRNVRGHNPLGLMYHDASRWGLTLQTYVQLTMLDR
HTRPQVSSVRLMERSIHSARYIFVENLYRSGKMPEVDYVVLSEWFDWILRNMDVSVDLIV
YLRTNPETCYQRLKKRCREEEKVIPLEYLEAIHHLHEEWLIKGSLFPMAAPVLVIEADHH
MERMLELFEQNRDRILTPENRKHCP
GenBank ID Protein 290656936
UniProtKB/Swiss-Prot ID O00142
UniProtKB/Swiss-Prot Entry Name KITM_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_004614.4
GeneCard ID TK2
GenAtlas ID TK2
HGNC ID HGNC:11831
References
General References
  1. Martin J, Han C, Gordon LA, Terry A, Prabhakar S, She X, Xie G, Hellsten U, Chan YM, Altherr M, Couronne O, Aerts A, Bajorek E, Black S, Blumer H, Branscomb E, Brown NC, Bruno WJ, Buckingham JM, Callen DF, Campbell CS, Campbell ML, Campbell EW, Caoile C, Challacombe JF, Chasteen LA, Chertkov O, Chi HC, Christensen M, Clark LM, Cohn JD, Denys M, Detter JC, Dickson M, Dimitrijevic-Bussod M, Escobar J, Fawcett JJ, Flowers D, Fotopulos D, Glavina T, Gomez M, Gonzales E, Goodstein D, Goodwin LA, Grady DL, Grigoriev I, Groza M, Hammon N, Hawkins T, Haydu L, Hildebrand CE, Huang W, Israni S, Jett J, Jewett PB, Kadner K, Kimball H, Kobayashi A, Krawczyk MC, Leyba T, Longmire JL, Lopez F, Lou Y, Lowry S, Ludeman T, Manohar CF, Mark GA, McMurray KL, Meincke LJ, Morgan J, Moyzis RK, Mundt MO, Munk AC, Nandkeshwar RD, Pitluck S, Pollard M, Predki P, Parson-Quintana B, Ramirez L, Rash S, Retterer J, Ricke DO, Robinson DL, Rodriguez A, Salamov A, Saunders EH, Scott D, Shough T, Stallings RL, Stalvey M, Sutherland RD, Tapia R, Tesmer JG, Thayer N, Thompson LS, Tice H, Torney DC, Tran-Gyamfi M, Tsai M, Ulanovsky LE, Ustaszewska A, Vo N, White PS, Williams AL, Wills PL, Wu JR, Wu K, Yang J, Dejong P, Bruce D, Doggett NA, Deaven L, Schmutz J, Grimwood J, Richardson P, Rokhsar DS, Eichler EE, Gilna P, Lucas SM, Myers RM, Rubin EM, Pennacchio LA: The sequence and analysis of duplication-rich human chromosome 16. Nature. 2004 Dec 23;432(7020):988-94. [PubMed:15616553 ]
  2. Wang L, Limongelli A, Vila MR, Carrara F, Zeviani M, Eriksson S: Molecular insight into mitochondrial DNA depletion syndrome in two patients with novel mutations in the deoxyguanosine kinase and thymidine kinase 2 genes. Mol Genet Metab. 2005 Jan;84(1):75-82. [PubMed:15639197 ]
  3. Johansson M, Karlsson A: Cloning of the cDNA and chromosome localization of the gene for human thymidine kinase 2. J Biol Chem. 1997 Mar 28;272(13):8454-8. [PubMed:9079672 ]
  4. Wang L, Munch-Petersen B, Herrstrom Sjoberg A, Hellman U, Bergman T, Jornvall H, Eriksson S: Human thymidine kinase 2: molecular cloning and characterisation of the enzyme activity with antiviral and cytostatic nucleoside substrates. FEBS Lett. 1999 Jan 25;443(2):170-4. [PubMed:9989599 ]
  5. Saada A, Shaag A, Mandel H, Nevo Y, Eriksson S, Elpeleg O: Mutant mitochondrial thymidine kinase in mitochondrial DNA depletion myopathy. Nat Genet. 2001 Nov;29(3):342-4. [PubMed:11687801 ]
  6. Mancuso M, Salviati L, Sacconi S, Otaegui D, Camano P, Marina A, Bacman S, Moraes CT, Carlo JR, Garcia M, Garcia-Alvarez M, Monzon L, Naini AB, Hirano M, Bonilla E, Taratuto AL, DiMauro S, Vu TH: Mitochondrial DNA depletion: mutations in thymidine kinase gene with myopathy and SMA. Neurology. 2002 Oct 22;59(8):1197-202. [PubMed:12391347 ]
  7. Tulinius M, Moslemi AR, Darin N, Holme E, Oldfors A: Novel mutations in the thymidine kinase 2 gene (TK2) associated with fatal mitochondrial myopathy and mitochondrial DNA depletion. Neuromuscul Disord. 2005 Jun;15(6):412-5. [PubMed:15907288 ]