| In D-loop: 40 years of mitochondrial 7S DNA TJ Nicholls, M Minczuk Experimental gerontology 56, 175-181, 2014 | 338 | 2014 |
| Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease C Kornblum, TJ Nicholls, TB Haack, S Schöler, V Peeva, K Danhauser, ... Nature genetics 45 (2), 214-219, 2013 | 261 | 2013 |
| ELAC2 mutations cause a mitochondrial RNA processing defect associated with hypertrophic cardiomyopathy TB Haack, R Kopajtich, P Freisinger, T Wieland, J Rorbach, TJ Nicholls, ... The American Journal of Human Genetics 93 (2), 211-223, 2013 | 160 | 2013 |
| Mutations in GTPBP3 cause a mitochondrial translation defect associated with hypertrophic cardiomyopathy, lactic acidosis, and encephalopathy R Kopajtich, TJ Nicholls, J Rorbach, MD Metodiev, P Freisinger, H Mandel, ... The American Journal of Human Genetics 95 (6), 708-720, 2014 | 142 | 2014 |
| Topoisomerase 3α is required for decatenation and segregation of human mtDNA TJ Nicholls, CA Nadalutti, E Motori, EW Sommerville, GS Gorman, S Basu, ... Molecular cell 69 (1), 9-23. e6, 2018 | 136 | 2018 |
| MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome J Rorbach, P Boesch, PA Gammage, TJJ Nicholls, SF Pearce, D Patel, ... Molecular biology of the cell 25 (17), 2542-2555, 2014 | 130 | 2014 |
| PDE12 removes mitochondrial RNA poly (A) tails and controls translation in human mitochondria J Rorbach, TJJ Nicholls, M Minczuk Nucleic acids research 39 (17), 7750-7763, 2011 | 126 | 2011 |
| Mitochondrial transcript maturation and its disorders L Van Haute, SF Pearce, CA Powell, AR D’Souza, TJ Nicholls, M Minczuk Journal of inherited metabolic disease 38, 655-680, 2015 | 98 | 2015 |
| Linear mtDNA fragments and unusual mtDNA rearrangements associated with pathological deficiency of MGME1 exonuclease TJ Nicholls, G Zsurka, V Peeva, S Schöler, RJ Szczesny, D Cysewski, ... Human molecular genetics 23 (23), 6147-6162, 2014 | 87 | 2014 |
| Nuclear-encoded factors involved in post-transcriptional processing and modification of mitochondrial tRNAs in human disease CA Powell, TJ Nicholls, M Minczuk Frontiers in genetics 6, 79, 2015 | 78 | 2015 |
| The maintenance of mitochondrial DNA integrity and dynamics by mitochondrial membranes J Chapman, YS Ng, TJ Nicholls Life 10 (9), 164, 2020 | 70 | 2020 |
| The mitochondrial single-stranded DNA binding protein is essential for initiation of mtDNA replication M Jiang, X Xie, X Zhu, S Jiang, D Milenkovic, J Misic, Y Shi, N Tandukar, ... Science advances 7 (27), eabf8631, 2021 | 68 | 2021 |
| Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria S Matic, M Jiang, TJ Nicholls, JP Uhler, C Dirksen-Schwanenland, ... Nature communications 9 (1), 1202, 2018 | 63 | 2018 |
| MGME1 processes flaps into ligatable nicks in concert with DNA polymerase γ during mtDNA replication JP Uhler, C Thörn, TJ Nicholls, S Matic, D Milenkovic, CM Gustafsson, ... Nucleic acids research 44 (12), 5861-5871, 2016 | 61 | 2016 |
| Separating and segregating the human mitochondrial genome TJ Nicholls, CM Gustafsson Trends in Biochemical Sciences 43 (11), 869-881, 2018 | 59 | 2018 |
| Mitochondria: mitochondrial RNA metabolism and human disease TJ Nicholls, J Rorbach, M Minczuk The international journal of biochemistry & cell biology 45 (4), 845-849, 2013 | 49 | 2013 |
| Mitochondrially-targeted APOBEC1 is a potent mtDNA mutator affecting mitochondrial function and organismal fitness in Drosophila S Andreazza, CL Samstag, A Sanchez-Martinez, E Fernandez-Vizarra, ... Nature communications 10 (1), 3280, 2019 | 32 | 2019 |
| Controlling the topology of mammalian mitochondrial DNA KE Menger, A Rodríguez-Luis, J Chapman, TJ Nicholls Open biology 11 (9), 210168, 2021 | 31 | 2021 |
| Dinucleotide degradation by REXO2 maintains promoter specificity in mammalian mitochondria TJ Nicholls, H Spåhr, S Jiang, SJ Siira, C Koolmeister, S Sharma, ... Molecular cell 76 (5), 784-796. e6, 2019 | 31 | 2019 |
| The human mitochondrial genome contains a second light strand promoter BG Tan, CD Mutti, Y Shi, X Xie, X Zhu, P Silva-Pinheiro, KE Menger, ... Molecular Cell 82 (19), 3646-3660. e9, 2022 | 27 | 2022 |
