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Disease causing mutation (P178L) in mitochondrial transcription factor A results in impaired mitochondrial transcription initiation.
Mehmedović M, Martucci M, Spåhr H, Ishak L, Mishra A, Sanchez-Sandoval ME, Pardo-Hernández C, Peter B, van den Wildenberg SM, Falkenberg M, Farge G. Mehmedović M, et al. Among authors: spahr h. Biochim Biophys Acta Mol Basis Dis. 2022 Oct 1;1868(10):166467. doi: 10.1016/j.bbadis.2022.166467. Epub 2022 Jun 15. Biochim Biophys Acta Mol Basis Dis. 2022. PMID: 35716868 Free article.
MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals.
Wredenberg A, Lagouge M, Bratic A, Metodiev MD, Spåhr H, Mourier A, Freyer C, Ruzzenente B, Tain L, Grönke S, Baggio F, Kukat C, Kremmer E, Wibom R, Polosa PL, Habermann B, Partridge L, Park CB, Larsson NG. Wredenberg A, et al. Among authors: spahr h. PLoS Genet. 2013;9(1):e1003178. doi: 10.1371/journal.pgen.1003178. Epub 2013 Jan 3. PLoS Genet. 2013. PMID: 23300484 Free PMC article.
A human mitochondrial poly(A) polymerase mutation reveals the complexities of post-transcriptional mitochondrial gene expression.
Wilson WC, Hornig-Do HT, Bruni F, Chang JH, Jourdain AA, Martinou JC, Falkenberg M, Spåhr H, Larsson NG, Lewis RJ, Hewitt L, Baslé A, Cross HE, Tong L, Lebel RR, Crosby AH, Chrzanowska-Lightowlers ZM, Lightowlers RN. Wilson WC, et al. Among authors: spahr h. Hum Mol Genet. 2014 Dec 1;23(23):6345-55. doi: 10.1093/hmg/ddu352. Epub 2014 Jul 9. Hum Mol Genet. 2014. PMID: 25008111 Free PMC article.
Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial nucleoid.
Kukat C, Davies KM, Wurm CA, Spåhr H, Bonekamp NA, Kühl I, Joos F, Polosa PL, Park CB, Posse V, Falkenberg M, Jakobs S, Kühlbrandt W, Larsson NG. Kukat C, et al. Among authors: spahr h. Proc Natl Acad Sci U S A. 2015 Sep 8;112(36):11288-93. doi: 10.1073/pnas.1512131112. Epub 2015 Aug 24. Proc Natl Acad Sci U S A. 2015. PMID: 26305956 Free PMC article.
Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria.
Nicholls TJ, Spåhr H, Jiang S, Siira SJ, Koolmeister C, Sharma S, Kauppila JHK, Jiang M, Kaever V, Rackham O, Chabes A, Falkenberg M, Filipovska A, Larsson NG, Gustafsson CM. Nicholls TJ, et al. Among authors: spahr h. Mol Cell. 2019 Dec 5;76(5):784-796.e6. doi: 10.1016/j.molcel.2019.09.010. Epub 2019 Oct 3. Mol Cell. 2019. PMID: 31588022 Free PMC article.
55 results