Zobrazeno 1 - 10
of 334
pro vyhledávání: '"J. Battersby"'
Autor:
Taru Hilander, Ryan Awadhpersad, Geoffray Monteuuis, Krystyna L. Broda, Max Pohjanpelto, Elizabeth Pyman, Sachin Kumar Singh, Tuula A. Nyman, Isabelle Crevel, Robert W. Taylor, Ann Saada, Diego Balboa, Brendan J. Battersby, Christopher B. Jackson, Christopher J. Carroll
Publikováno v:
iScience, Vol 27, Iss 7, Pp 110185- (2024)
Summary: Mitochondrial ribosomes (mitoribosomes) have undergone substantial evolutionary structural remodeling accompanied by loss of ribosomal RNA, while acquiring unique protein subunits located on the periphery. We generated CRISPR-mediated knocko
Externí odkaz:
https://doaj.org/article/eb4d883dcd61474084b359d3f09df2e4
Autor:
Katarin Gorski, Christopher B. Jackson, Tuula A. Nyman, Veronika Rezov, Brendan J. Battersby, Anna-Elina Lehesjoki
Publikováno v:
Frontiers in Molecular Neuroscience, Vol 16 (2023)
The involvement of mitochondrial dysfunction in cystatin B (CSTB) deficiency has been suggested, but its role in the onset of neurodegeneration, myoclonus, and ataxia in the CSTB-deficient mouse model (Cstb−/−) is yet unknown. CSTB is an inhibito
Externí odkaz:
https://doaj.org/article/42d41ce53b244a058994c702cd443e36
Autor:
Kimberly A. Jett, Zakery N. Baker, Amzad Hossain, Aren Boulet, Paul A. Cobine, Sagnika Ghosh, Philip Ng, Orhan Yilmaz, Kris Barreto, John DeCoteau, Karen Mochoruk, George N. Ioannou, Christopher Savard, Sai Yuan, Osama H.M.H. Abdalla, Christopher Lowden, Byung-Eun Kim, Hai-Ying Mary Cheng, Brendan J. Battersby, Vishal M. Gohil, Scot C. Leary
Publikováno v:
The Journal of Clinical Investigation, Vol 133, Iss 1 (2023)
Signaling circuits crucial to systemic physiology are widespread, yet uncovering their molecular underpinnings remains a barrier to understanding the etiology of many metabolic disorders. Here, we identified a copper-linked signaling circuit activate
Externí odkaz:
https://doaj.org/article/018c1e9c28eb4a92a02a5abcb7afd106
Autor:
Cecilia Mancini, Eriola Hoxha, Luisa Iommarini, Alessandro Brussino, Uwe Richter, Francesca Montarolo, Claudia Cagnoli, Roberta Parolisi, Diana Iulia Gondor Morosini, Valentina Nicolò, Francesca Maltecca, Luisa Muratori, Giulia Ronchi, Stefano Geuna, Francesca Arnaboldi, Elena Donetti, Elisa Giorgio, Simona Cavalieri, Eleonora Di Gregorio, Elisa Pozzi, Marta Ferrero, Evelise Riberi, Giorgio Casari, Fiorella Altruda, Emilia Turco, Giuseppe Gasparre, Brendan J. Battersby, Anna Maria Porcelli, Enza Ferrero, Alfredo Brusco, Filippo Tempia
Publikováno v:
Neurobiology of Disease, Vol 124, Iss , Pp 14-28 (2019)
Spinocerebellar ataxia 28 is an autosomal dominant neurodegenerative disorder caused by missense mutations affecting the proteolytic domain of AFG3L2, a major component of the mitochondrial m-AAA protease. However, little is known of the underlying p
Externí odkaz:
https://doaj.org/article/fb3744284a404bcf85efa0b2b018e6b8
Autor:
Katarin Gorski, Albert Spoljaric, Tuula A. Nyman, Kai Kaila, Brendan J. Battersby, Anna-Elina Lehesjoki
Publikováno v:
Frontiers in Molecular Neuroscience, Vol 13 (2020)
Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is a neurodegenerative disorder caused by loss-of-function mutations in the cystatin B (CSTB) gene. Progression of the clinical symptoms in EPM1 patients, including stimulus-sensitive
Externí odkaz:
https://doaj.org/article/f25b3b2f46794c56a9d29d0251530174
Autor:
Kyle Thompson, Nicole Mai, Monika Oláhová, Filippo Scialó, Luke E Formosa, David A Stroud, Madeleine Garrett, Nichola Z Lax, Fiona M Robertson, Cristina Jou, Andres Nascimento, Carlos Ortez, Cecilia Jimenez‐Mallebrera, Steven A Hardy, Langping He, Garry K Brown, Paula Marttinen, Robert McFarland, Alberto Sanz, Brendan J Battersby, Penelope E Bonnen, Michael T Ryan, Zofia MA Chrzanowska‐Lightowlers, Robert N Lightowlers, Robert W Taylor
Publikováno v:
EMBO Molecular Medicine, Vol 10, Iss 11, Pp 1-13 (2018)
Abstract OXA1, the mitochondrial member of the YidC/Alb3/Oxa1 membrane protein insertase family, is required for the assembly of oxidative phosphorylation complexes IV and V in yeast. However, depletion of human OXA1 (OXA1L) was previously reported t
Externí odkaz:
https://doaj.org/article/0d713560574b49dfa1120e2d8edbb05c
Autor:
Uwe Richter, Molly E. Evans, Wesley C. Clark, Paula Marttinen, Eric A. Shoubridge, Anu Suomalainen, Anna Wredenberg, Anna Wedell, Tao Pan, Brendan J. Battersby
Publikováno v:
Nature Communications, Vol 9, Iss 1, Pp 1-11 (2018)
Mutations in mitochondrially-encoded tRNA genes can lead to mitochondrial disorders. Here the authors use next generation RNA sequencing to reveal the role of a N1 -methyladenosine modification in tRNALys MERR patients for translation elongation and
Externí odkaz:
https://doaj.org/article/6476c28b9ae34dcfb308b5409a438859
Autor:
Nick J. Battersby, Hannah Wright, Tahseen Qureshi, Thomas Parker, Nuno Figueiredo, Amjad Parvaiz
Publikováno v:
Journal of Laparoendoscopic & Advanced Surgical Techniques. 32:938-947
Autor:
Nick J. Battersby, Mit Dattani, Sheela Rao, David Cunningham, Diana Tait, Richard Adams, Brendan J. Moran, Shelize Khakoo, Paris Tekkis, Shahnawaz Rasheed, Alex Mirnezami, Philip Quirke, Nicholas P. West, Iris Nagtegaal, Irene Chong, Anguraj Sadanandam, Nicola Valeri, Karen Thomas, Michelle Frost, Gina Brown
Publikováno v:
Trials, Vol 18, Iss 1, Pp 1-14 (2017)
Abstract Background Pre-operative chemoradiotherapy (CRT) for MRI-defined, locally advanced rectal cancer is primarily intended to reduce local recurrence rates by downstaging tumours, enabling an improved likelihood of curative resection. However, i
Externí odkaz:
https://doaj.org/article/d33ef947bcf149739383193bb09a5f50
Autor:
Ena Tomoda, Asuteka Nagao, Yuki Shirai, Kana Asano, Takeo Suzuki, Brendan J Battersby, Tsutomu Suzuki
Publikováno v:
Nucleic Acids Research.
Mutations in mitochondrial (mt-)tRNAs frequently cause mitochondrial dysfunction. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), and myoclonus epilepsy associated with ragged red fibers (MERRF) are major cl