What cost mitochondria? The maintenance of functional mitochondrial DNA within and across generations

Por Aanen, Duur K., Spelbrink, Johannes N., Beekman, Madeleine
Publicado em 2014

Quality matters: how does mitochondrial network dynamics and quality control impact on mtDNA integrity?

Por Busch, Karin B., Kowald, Axel, Spelbrink, Johannes N.
Publicado em 2014

A New Splice Variant of the Mouse SIRT3 Gene Encodes the Mitochondrial Precursor Protein

Por Cooper, Helen M., Huang, Jing-Yi, Verdin, Eric, Spelbrink, Johannes N.
Publicado em 2009

Replication factors transiently associate with mtDNA at the mitochondrial inner membrane to facilitate replication

Por Rajala, Nina, Gerhold, Joachim M., Martinsson, Peter, Klymov, Alexey, Spelbrink, Johannes N.
Publicado em 2014

The mitochondrial outer-membrane location of the EXD2 exonuclease contradicts its direct role in nuclear DNA repair

Por Hensen, Fenna, Moretton, Amandine, van Esveld, Selma, Farge, Géraldine, Spelbrink, Johannes N.
Publicado no Sci Rep (2018)

Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes

Por Wanrooij, Sjoerd, Goffart, Steffi, Pohjoismäki, Jaakko L.O., Yasukawa, Takehiro, Spelbrink, Johannes N.
Publicado em 2007

Twinkle mutations associated with autosomal dominant progressive external ophthalmoplegia lead to impaired helicase function and in vivo mtDNA replication stalling

Por Goffart, Steffi, Cooper, Helen M., Tyynismaa, Henna, Wanrooij, Sjoerd, Suomalainen, Anu, Spelbrink, Johannes N.
Publicado em 2009

Twinkle and POLG defects enhance age-dependent accumulation of mutations in the control region of mtDNA

Por Wanrooij, Sjoerd, Luoma, Petri, van Goethem, Gert, van Broeckhoven, Christine, Suomalainen, Anu, Spelbrink, Johannes N.
Publicado em 2004

Whole Cell Formaldehyde Cross-Linking Simplifies Purification of Mitochondrial Nucleoids and Associated Proteins Involved in Mitochondrial Gene Expression

Por Rajala, Nina, Hensen, Fenna, Wessels, Hans J. C. T., Ives, Daniel, Gloerich, Jolein, Spelbrink, Johannes N.
Publicado no PLoS One (2015)

Composition and Dynamics of Human Mitochondrial Nucleoids

Por Garrido, Nuria, Griparic, Lorena, Jokitalo, Eija, Wartiovaara, Jorma, van der Bliek, Alexander M., Spelbrink, Johannes N.
Publicado em 2003

Mitochondrial RNA granules are critically dependent on mtDNA replication factors Twinkle and mtSSB

Por Hensen, Fenna, Potter, Alisa, van Esveld, Selma L, Tarrés-Solé, Aleix, Chakraborty, Arka, Solà, Maria, Spelbrink, Johannes N
Publicado no Nucleic Acids Res (2019)

Alterations to the expression level of mitochondrial transcription factor A, TFAM, modify the mode of mitochondrial DNA replication in cultured human cells

Por Pohjoismäki, Jaakko L. O., Wanrooij, Sjoerd, Hyvärinen, Anne K., Goffart, Steffi, Holt, Ian J., Spelbrink, Johannes N., Jacobs, Howard T.
Publicado em 2006

A Combined Mass Spectrometry and Data Integration Approach to Predict the Mitochondrial Poly(A) RNA Interacting Proteome

Por van Esveld, Selma L., Cansız-Arda, Şirin, Hensen, Fenna, van der Lee, Robin, Huynen, Martijn A., Spelbrink, Johannes N.
Publicado no Front Cell Dev Biol (2019)

Mutant mitochondrial helicase Twinkle causes multiple mtDNA deletions and a late-onset mitochondrial disease in mice

Por Tyynismaa, Henna, Mjosund, Katja Peltola, Wanrooij, Sjoerd, Lappalainen, Ilse, Ylikallio, Emil, Jalanko, Anu, Spelbrink, Johannes N., Paetau, Anders, Suomalainen, Anu
Publicado em 2005

Human Dna2 Is a Nuclear and Mitochondrial DNA Maintenance Protein

Por Duxin, Julien P., Dao, Benjamin, Martinsson, Peter, Rajala, Nina, Guittat, Lionel, Campbell, Judith L., Spelbrink, Johannes N., Stewart, Sheila A.
Publicado em 2009

DNA Sequences Proximal to Human Mitochondrial DNA Deletion Breakpoints Prevalent in Human Disease Form G-quadruplexes, a Class of DNA Structures Inefficiently Unwound by the Mitoch...

Por Bharti, Sanjay Kumar, Sommers, Joshua A., Zhou, Jun, Kaplan, Daniel L., Spelbrink, Johannes N., Mergny, Jean-Louis, Brosh, Robert M.
Publicado em 2014

Somatic mtDNA mutations cause aging phenotypes without affecting reactive oxygen species production

Por Trifunovic, Aleksandra, Hansson, Anna, Wredenberg, Anna, Rovio, Anja T., Dufour, Eric, Khvorostov, Ivan, Spelbrink, Johannes N., Wibom, Rolf, Jacobs, Howard T., Larsson, Nils-Göran
Publicado em 2005

Transcript availability dictates the balance between strand-asynchronous and strand-coupled mitochondrial DNA replication

Por Cluett, Tricia J, Akman, Gokhan, Reyes, Aurelio, Kazak, Lawrence, Mitchell, Alice, Wood, Stuart R, Spinazzola, Antonella, Spelbrink, Johannes N, Holt, Ian J
Publicado no Nucleic Acids Res (2018)

The mitochondrial transcription termination factor mTERF modulates replication pausing in human mitochondrial DNA

Por Hyvärinen, Anne K., Pohjoismäki, Jaakko L. O., Reyes, Aurelio, Wanrooij, Sjoerd, Yasukawa, Takehiro, Karhunen, Pekka J., Spelbrink, Johannes N., Holt, Ian J., Jacobs, Howard T.
Publicado em 2007

Human Mitochondrial DNA-Protein Complexes Attach to a Cholesterol-Rich Membrane Structure

Por Gerhold, Joachim M., Cansiz-Arda, Şirin, Lõhmus, Madis, Engberg, Oskar, Reyes, Aurelio, van Rennes, Helga, Sanz, Alberto, Holt, Ian J., Cooper, Helen M., Spelbrink, Johannes N.
Publicado no Sci Rep (2015)