Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism.

doi:10.1016/j.cmet.2016.01.019

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Article

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism.

Cell metabolism - 12 Apr 2016

Nikkanen Joni, Forsström Saara, Euro Liliya, Paetau Ilse, Kohnz Rebecca A, Wang Liya, Chilov Dmitri, Viinamäki Jenni, Roivainen Anne, Marjamäki Päivi, Liljenbäck Heidi, Ahola Sofia, Buzkova Jana, Terzioglu Mügen, Khan Nahid A, Pirnes-Karhu Sini, Paetau Anders, Lönnqvist Tuula, Sajantila Antti, Isohanni Pirjo, Tyynismaa Henna, Nomura Daniel K, Battersby Brendan J, Velagapudi Vidya, Carroll Christopher J, Suomalainen Anu

Abstract excerpt

Mitochondrial dysfunction affects cellular energy metabolism, but less is known about the consequences for cytoplasmic biosynthetic reactions. We report that mtDNA replication disorders caused by TWINKLE mutations-mitochondrial myopathy (MM) and infantile onset spinocerebellar ataxia (IOSCA)-remodel cellular dNTP pools in mice. MM muscle shows tissue-specific induction of the mitochondrial folate cycle, purine...

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PMID: 26924217
DOI: 10.1016/j.cmet.2016.01.019

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

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