Quality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness

por Richter, Uwe, Lahtinen, Taina, Marttinen, Paula, Suomi, Fumi, Battersby, Brendan J.
Publicado 2015

No recombination of mtDNA after heteroplasmy for 50 generations in the mouse maternal germline

por Hagström, Erik, Freyer, Christoph, Battersby, Brendan J., Stewart, James B., Larsson, Nils-Göran
Publicado 2014

Translation of MT-ATP6 pathogenic variants reveals distinct regulatory consequences from the co-translational quality control of mitochondrial protein synthesis

por Ng, Kah Ying, Richter, Uwe, Jackson, Christopher B, Seneca, Sara, Battersby, Brendan J
Publicado 2021

Restoration of mitochondrial function through activation of hypomodified tRNAs with pathogenic mutations associated with mitochondrial diseases

por Tomoda, Ena, Nagao, Asuteka, Shirai, Yuki, Asano, Kana, Suzuki, Takeo, Battersby, Brendan J, Suzuki, Tsutomu
Publicado 2023

Quantitative Changes in the Mitochondrial Proteome of Cerebellar Synaptosomes From Preclinical Cystatin B-Deficient Mice

por Gorski, Katarin, Spoljaric, Albert, Nyman, Tuula A., Kaila, Kai, Battersby, Brendan J., Lehesjoki, Anna-Elina
Publicado 2020

Progressive mitochondrial dysfunction in cerebellar synaptosomes of cystatin B-deficient mice

por Gorski, Katarin, Jackson, Christopher B., Nyman, Tuula A., Rezov, Veronika, Battersby, Brendan J., Lehesjoki, Anna-Elina
Publicado 2023

RNA modification landscape of the human mitochondrial tRNA(Lys) regulates protein synthesis

por Richter, Uwe, Evans, Molly E., Clark, Wesley C., Marttinen, Paula, Shoubridge, Eric A., Suomalainen, Anu, Wredenberg, Anna, Wedell, Anna, Pan, Tao, Battersby, Brendan J.
Publicado 2018

Gimap3 Regulates Tissue-Specific Mitochondrial DNA Segregation

por Jokinen, Riikka, Marttinen, Paula, Sandell, Helen Katarin, Manninen, Tuula, Teerenhovi, Heli, Wai, Timothy, Teoli, Daniella, Loredo-Osti, J. C., Shoubridge, Eric A., Battersby, Brendan J.
Publicado 2010

Nonstop mRNAs generate a ground state of mitochondrial gene expression noise

por Ng, Kah Ying, Lutfullahoglu Bal, Guleycan, Richter, Uwe, Safronov, Omid, Paulin, Lars, Dunn, Cory D., Paavilainen, Ville O., Richer, Julie, Newman, William G., Taylor, Robert W., Battersby, Brendan J.
Publicado 2022

Mitochondrial stress response triggered by defects in protein synthesis quality control

por Richter, Uwe, Ng, Kah Ying, Suomi, Fumi, Marttinen, Paula, Turunen, Taina, Jackson, Christopher, Suomalainen, Anu, Vihinen, Helena, Jokitalo, Eija, Nyman, Tuula A, Isokallio, Marita A, Stewart, James B, Mancini, Cecilia, Brusco, Alfredo, Seneca, Sara, Lombès, Anne, Taylor, Robert W, Battersby, Brendan J
Publicado 2019

Mitochondrial dysfunction reactivates α-fetoprotein expression that drives copper-dependent immunosuppression in mitochondrial disease models

por Jett, Kimberly A., Baker, Zakery N., Hossain, Amzad, Boulet, Aren, Cobine, Paul A., Ghosh, Sagnika, Ng, Philip, Yilmaz, Orhan, Barreto, Kris, DeCoteau, John, Mochoruk, Karen, Ioannou, George N., Savard, Christopher, Yuan, Sai, Abdalla, Osama H.M.H., Lowden, Christopher, Kim, Byung-Eun, Cheng, Hai-Ying Mary, Battersby, Brendan J., Gohil, Vishal M., Leary, Scot C.
Publicado 2023

OXA1L mutations cause mitochondrial encephalopathy and a combined oxidative phosphorylation defect

por Thompson, Kyle, Mai, Nicole, Oláhová, Monika, Scialó, Filippo, Formosa, Luke E, Stroud, David A, Garrett, Madeleine, Lax, Nichola Z, Robertson, Fiona M, Jou, Cristina, Nascimento, Andres, Ortez, Carlos, Jimenez‐Mallebrera, Cecilia, Hardy, Steven A, He, Langping, Brown, Garry K, Marttinen, Paula, McFarland, Robert, Sanz, Alberto, Battersby, Brendan J, Bonnen, Penelope E, Ryan, Michael T, Chrzanowska‐Lightowlers, Zofia MA, Lightowlers, Robert N, Taylor, Robert W
Publicado 2018

Bi-allelic variants in the mitochondrial RNase P subunit PRORP cause mitochondrial tRNA processing defects and pleiotropic multisystem presentations

por Hochberg, Irit, Demain, Leigh A.M., Richer, Julie, Thompson, Kyle, Urquhart, Jill E., Rea, Alessandro, Pagarkar, Waheeda, Rodríguez-Palmero, Agustí, Schlüter, Agatha, Verdura, Edgard, Pujol, Aurora, Quijada-Fraile, Pilar, Amberger, Albert, Deutschmann, Andrea J., Demetz, Sandra, Gillespie, Meredith, Belyantseva, Inna A., McMillan, Hugh J., Barzik, Melanie, Beaman, Glenda M., Motha, Reeya, Ng, Kah Ying, O’Sullivan, James, Williams, Simon G., Bhaskar, Sanjeev S., Lawrence, Isabella R., Jenkinson, Emma M., Zambonin, Jessica L., Blumenfeld, Zeev, Yalonetsky, Sergey, Oerum, Stephanie, Rossmanith, Walter, Yue, Wyatt W., Zschocke, Johannes, Munro, Kevin J., Battersby, Brendan J., Friedman, Thomas B., Taylor, Robert W., O’Keefe, Raymond T., Newman, William G.
Publicado 2021