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Thymidine kinase 2 (TK2) is a mitochondrial enzyme that catalyzes the phosphorylation of pyrimidine deoxynucleosides, specifically thymidine, deoxycytidine, and deoxyuridine, to their corresponding monophosphate forms. This is essential for maintaining adequate pools of deoxynucleotide triphosphates (dNTPs) required for mitochondrial DNA (mtDNA) replication and repair. Mutations in the TK2 gene cause thymidine kinase 2 deficiency, leading to a myopathic form of mitochondrial DNA depletion syndrome.
Phosphorylates pyrimidine deoxynucleosides to their monophosphate forms, providing building blocks for mtDNA replication and repair.
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