Mitochondrial biology and dysfunction in secondary mitochondrial disease

por Baker, Megan J., Crameri, Jordan J., Thorburn, David R., Frazier, Ann E., Stojanovski, Diana
Publicado 2022

Modelling Mitochondrial Disease in Human Pluripotent Stem Cells: What Have We Learned?

por McKnight, Cameron L., Low, Yau Chung, Elliott, David A., Thorburn, David R., Frazier, Ann E.
Publicado 2021

Neuronal and astrocyte dysfunction diverges from embryonic fibroblasts in the Ndufs4(fky/fky) mouse

por Bird, Matthew J., Wijeyeratne, Xiaonan W., Komen, Jasper C., Laskowski, Adrienne, Ryan, Michael T., Thorburn, David R., Frazier, Ann E.
Publicado 2014

Mdm38 interacts with ribosomes and is a component of the mitochondrial protein export machinery

por Frazier, Ann E., Taylor, Rebecca D., Mick, David U., Warscheid, Bettina, Stoepel, Nadine, Meyer, Helmut E., Ryan, Michael T., Guiard, Bernard, Rehling, Peter
Publicado 2006

A J-protein is an essential subunit of the presequence translocase–associated protein import motor of mitochondria

por Truscott, Kaye N., Voos, Wolfgang, Frazier, Ann E., Lind, Maria, Li, Yanfeng, Geissler, Andreas, Dudek, Jan, Müller, Hanne, Sickmann, Albert, Meyer, Helmut E., Meisinger, Chris, Guiard, Bernard, Rehling, Peter, Pfanner, Nikolaus
Publicado 2003

The TIM22 complex mediates the import of sideroflexins and is required for efficient mitochondrial one-carbon metabolism

por Jackson, Thomas D., Hock, Daniella H., Fujihara, Kenji M., Palmer, Catherine S., Frazier, Ann E., Low, Yau C., Kang, Yilin, Ang, Ching-Seng, Clemons, Nicholas J., Thorburn, David R., Stroud, David A., Stojanovski, Diana
Publicado 2021

Mitochondrial OXA Translocase Plays a Major Role in Biogenesis of Inner-Membrane Proteins

por Stiller, Sebastian B., Höpker, Jan, Oeljeklaus, Silke, Schütze, Conny, Schrempp, Sandra G., Vent-Schmidt, Jens, Horvath, Susanne E., Frazier, Ann E., Gebert, Natalia, van der Laan, Martin, Bohnert, Maria, Warscheid, Bettina, Pfanner, Nikolaus, Wiedemann, Nils
Publicado 2016

Functional Characterization of Friedreich Ataxia iPS-Derived Neuronal Progenitors and Their Integration in the Adult Brain

por Bird, Matthew J., Needham, Karina, Frazier, Ann E., van Rooijen, Jorien, Leung, Jessie, Hough, Shelley, Denham, Mark, Thornton, Matthew E., Parish, Clare L., Nayagam, Bryony A., Pera, Martin, Thorburn, David R., Thompson, Lachlan H., Dottori, Mirella
Publicado 2014

Sideroflexin 4 is a complex I assembly factor that interacts with the MCIA complex and is required for the assembly of the ND2 module

por Jackson, Thomas D., Crameri, Jordan J., Muellner-Wong, Linden, Frazier, Ann E., Palmer, Catherine S., Formosa, Luke E., Hock, Daniella H., Fujihara, Kenji M., Stait, Tegan, Sharpe, Alice J., Thorburn, David R., Ryan, Michael T., Stroud, David A., Stojanovski, Diana
Publicado 2022

Function of hTim8a in complex IV assembly in neuronal cells provides insight into pathomechanism underlying Mohr-Tranebjærg syndrome

por Kang, Yilin, Anderson, Alexander J, Jackson, Thomas Daniel, Palmer, Catherine S, De Souza, David P, Fujihara, Kenji M, Stait, Tegan, Frazier, Ann E, Clemons, Nicholas J, Tull, Deidreia, Thorburn, David R, McConville, Malcolm J, Ryan, Michael T, Stroud, David A, Stojanovski, Diana
Publicado 2019

Correction: Function of hTim8a in complex IV assembly in neuronal cells provides insight into pathomechanism underlying Mohr-Tranebjærg syndrome

por Kang, Yilin, Anderson, Alexander J, Jackson, Thomas Daniel, Palmer, Catherine S, De Souza, David P, Fujihara, Kenji M, Stait, Tegan, Frazier, Ann E, Clemons, Nicholas J, Tull, Deidreia, Thorburn, David R, McConville, Malcolm J, Ryan, Michael T, Stroud, David A, Stojanovski, Diana
Publicado 2020

High-intensity training induces non-stoichiometric changes in the mitochondrial proteome of human skeletal muscle without reorganisation of respiratory chain content

por Granata, Cesare, Caruana, Nikeisha J., Botella, Javier, Jamnick, Nicholas A., Huynh, Kevin, Kuang, Jujiao, Janssen, Hans A., Reljic, Boris, Mellett, Natalie A., Laskowski, Adrienne, Stait, Tegan L., Frazier, Ann E., Coughlan, Melinda T., Meikle, Peter J., Thorburn, David R., Stroud, David A., Bishop, David J.
Publicado 2021

Mutations in the UQCC1-Interacting Protein, UQCC2, Cause Human Complex III Deficiency Associated with Perturbed Cytochrome b Protein Expression

por Tucker, Elena J., Wanschers, Bas F. J., Szklarczyk, Radek, Mountford, Hayley S., Wijeyeratne, Xiaonan W., van den Brand, Mariël A. M., Leenders, Anne M., Rodenburg, Richard J., Reljić, Boris, Compton, Alison G., Frazier, Ann E., Bruno, Damien L., Christodoulou, John, Endo, Hitoshi, Ryan, Michael T., Nijtmans, Leo G., Huynen, Martijn A., Thorburn, David R.
Publicado 2013

ATAD3 gene cluster deletions cause cerebellar dysfunction associated with altered mitochondrial DNA and cholesterol metabolism

por Desai, Radha, Frazier, Ann E., Durigon, Romina, Patel, Harshil, Jones, Aleck W., Dalla Rosa, Ilaria, Lake, Nicole J., Compton, Alison G., Mountford, Hayley S., Tucker, Elena J., Mitchell, Alice L. R., Jackson, Deborah, Sesay, Abdul, Di Re, Miriam, van den Heuvel, Lambert P., Burke, Derek, Francis, David, Lunke, Sebastian, McGillivray, George, Mandelstam, Simone, Mochel, Fanny, Keren, Boris, Jardel, Claude, Turner, Anne M., Ian Andrews, P., Smeitink, Jan, Spelbrink, Johannes N., Heales, Simon J., Kohda, Masakazu, Ohtake, Akira, Murayama, Kei, Okazaki, Yasushi, Lombès, Anne, Holt, Ian J., Thorburn, David R., Spinazzola, Antonella
Publicado 2017