Zobrazeno 1 - 10
of 17
pro vyhledávání: '"Ann E. Frazier"'
Publikováno v:
Open Biology, Vol 12, Iss 12 (2022)
Mitochondrial diseases are a broad, genetically heterogeneous class of metabolic disorders characterized by deficits in oxidative phosphorylation (OXPHOS). Primary mitochondrial disease (PMD) defines pathologies resulting from mutation of mitochondri
Externí odkaz:
https://doaj.org/article/50fbab3eae4841b0976ca1bdaf33b93d
Autor:
Cesare Granata, Nikeisha J. Caruana, Javier Botella, Nicholas A. Jamnick, Kevin Huynh, Jujiao Kuang, Hans A. Janssen, Boris Reljic, Natalie A. Mellett, Adrienne Laskowski, Tegan L. Stait, Ann E. Frazier, Melinda T. Coughlan, Peter J. Meikle, David R. Thorburn, David A. Stroud, David J. Bishop
Publikováno v:
Nature Communications, Vol 12, Iss 1, Pp 1-18 (2021)
Exercise training can be therapeutic but how mitochondria respond remains unclear. Here, the authors use multiple omics techniques to reveal a complex network of non-stoichiometric mitochondrial adaptations that are prioritized or deprioritised durin
Externí odkaz:
https://doaj.org/article/bea2037780444510914f0ea9d4120a6f
Publikováno v:
International Journal of Molecular Sciences, Vol 22, Iss 14, p 7730 (2021)
Mitochondrial diseases disrupt cellular energy production and are among the most complex group of inherited genetic disorders. Affecting approximately 1 in 5000 live births, they are both clinically and genetically heterogeneous, and can be highly ti
Externí odkaz:
https://doaj.org/article/fc0ae808cbc344adb2efa1564e1e7fe2
Autor:
Matthew J. Bird, Xiaonan W. Wijeyeratne, Jasper C. Komen, Adrienne Laskowski, Michael T. Ryan, David R. Thorburn, Ann E. Frazier
Publikováno v:
Bioscience Reports, Vol 34, Iss 6, p e00151 (2014)
Mitochondrial dysfunction causes a range of early-onset neurological diseases and contributes to neurodegenerative conditions. The mechanisms of neurological damage however are poorly understood, as accessing relevant tissue from patients is difficul
Externí odkaz:
https://doaj.org/article/24a09d353dc54301b46476e31afb1d0c
Autor:
Daniella H. Hock, Alison G. Compton, Sumudu S.C. Amarasekera, Ann E. Frazier, Guy Helman, Nicole J. Lake, Liana N. Semcesen, Zornitza Stark, Monkol Lek, Cas Simons, John Christodoulou, David R. Thorburn, David A. Stroud
Publikováno v:
Pathology. 54:S17
Publikováno v:
International Journal of Molecular Sciences
International Journal of Molecular Sciences, Vol 22, Iss 7730, p 7730 (2021)
International Journal of Molecular Sciences, Vol 22, Iss 7730, p 7730 (2021)
Mitochondrial diseases disrupt cellular energy production and are among the most complex group of inherited genetic disorders. Affecting approximately 1 in 5000 live births, they are both clinically and genetically heterogeneous, and can be highly ti
Publikováno v:
Biochimica et Biophysica Acta (BBA) - General Subjects. 1840:1380-1392
Background The neuropathology of mitochondrial disease is well characterised. However, pathophysiological mechanisms at the level of biochemistry and cell biology are less clear. Progress in this area has been hampered by the limited accessibility of
Autor:
Inge Perschil, Martin van der Laan, Bettina Warscheid, Karina Wagner, David U. Mick, Ann E. Frazier, Magdalena Pawlas, Peter Rehling, Helmut E. Meyer
Publikováno v:
The EMBO Journal. 26:4347-4358
Cytochrome c oxidase (complex IV) of the respiratory chain is assembled from nuclear and mitochondrially-encoded subunits. Defects in the assembly process lead to severe human disorders such as Leigh syndrome. Shy1 is an assembly factor for complex I
Publikováno v:
ResearcherID
Most mitochondrial proteins are synthesized in the cytosol, translocated into the organelle and directed along specific sorting pathways. Over the past 20 years, >30 proteins have been identified as having key roles in mitochondrial protein import. R
Autor:
Albert Sickmann, Virginia Bilanchone, M G Cumsky, Kaye N. Truscott, Helmut E. Meyer, Yanfeng Li, Andreas Geissler, Ann E. Frazier, Wolfgang Voos, Chris Meisinger, Maria I. Lind, Peter Rehling, Jan Dudek, Bernard Guiard, Nikolaus Pfanner
Publikováno v:
Nature Structural & Molecular Biology. 11:226-233
Mitochondrial preproteins destined for the matrix are translocated by two channel-forming transport machineries, the translocase of the outer membrane and the presequence translocase of the inner membrane. The presequence translocase-associated prote