Product References
Multi-omics identifies large mitoribosomal subunit instability caused by pathogenic MRPL39 variants as a cause of pediatric onset mitochondrial disease.
Human molecular genetics
Amarasekera SSC,Hock DH,Lake NJ,Calvo SE,Grønborg SW,Krzesinski EI,Amor DJ,Fahey MC,Simons C,Wibrand F,Mootha VK,Lek M,Lunke S,Stark Z,Østergaard E,Christodoulou J,Thorburn DR,Stroud DA,Compton AG
Thu Jul 20 00:00:00 UTC 2023
Tetracycline-dependent inhibition of mitoribosome protein elongation in mitochondrial disease mutant cells suppresses IRE1α to promote cell survival.
bioRxiv : the preprint server for biology
Ronayne CT,Bennett CF,Perry EA,Kantorovich N,Puigserver P
Tue Mar 21 00:00:00 UTC 2023
Non-canonical phosphoglycerate dehydrogenase activity promotes liver cancer growth via mitochondrial translation and respiratory metabolism.
The EMBO journal
Shu Y,Hao Y,Feng J,Liu H,Li ST,Feng J,Jiang Z,Ye L,Zhou Y,Sun Y,Zhou Z,Wei H,Gao P,Zhang H,Sun L
Thu Dec 01 00:00:00 UTC 2022
Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan.
Aging cell
Richman TR,Ermer JA,Siira SJ,Kuznetsova I,Brosnan CA,Rossetti G,Baker J,Perks KL,Cserne Szappanos H,Viola HM,Gray N,Larance M,Hool LC,Zuryn S,Rackham O,Filipovska A
Thu Jul 01 00:00:00 UTC 2021
Defining the interactome of the human mitochondrial ribosome identifies SMIM4 and TMEM223 as respiratory chain assembly factors.
eLife
Dennerlein S,Poerschke S,Oeljeklaus S,Wang C,Richter-Dennerlein R,Sattmann J,Bauermeister D,Hanitsch E,Stoldt S,Langer T,Jakobs S,Warscheid B,Rehling P
Fri Dec 31 00:00:00 UTC 2021
Cardiolipin is required for membrane docking of mitochondrial ribosomes and protein synthesis.
Journal of cell science
Lee RG,Gao J,Siira SJ,Shearwood AM,Ermer JA,Hofferek V,Mathews JC,Zheng M,Reid GE,Rackham O,Filipovska A
Fri Jul 24 00:00:00 UTC 2020
Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation.
The EMBO journal
Ferreira N,Perks KL,Rossetti G,Rudler DL,Hughes LA,Ermer JA,Scott LH,Kuznetsova I,Richman TR,Narayana VK,Abudulai LN,Shearwood AJ,Cserne Szappanos H,Tull D,Yeoh GC,Hool LC,Filipovska A,Rackham O
Mon Dec 16 00:00:00 UTC 2019
MitoRibo-Tag Mice Provide a Tool for In Vivo Studies of Mitoribosome Composition.
Cell reports
Busch JD,Cipullo M,Atanassov I,Bratic A,Silva Ramos E,Schöndorf T,Li X,Pearce SF,Milenkovic D,Rorbach J,Larsson NG
Tue Nov 05 00:00:00 UTC 2019
Fidelity of translation initiation is required for coordinated respiratory complex assembly.
Science advances
Rudler DL,Hughes LA,Perks KL,Richman TR,Kuznetsova I,Ermer JA,Abudulai LN,Shearwood AJ,Viola HM,Hool LC,Siira SJ,Rackham O,Filipovska A
Sun Dec 01 00:00:00 UTC 2019
The human Obg protein GTPBP10 is involved in mitoribosomal biogenesis.
Nucleic acids research
Lavdovskaia E,Kolander E,Steube E,Mai MM,Urlaub H,Richter-Dennerlein R
Wed Sep 19 00:00:00 UTC 2018
The human Obg protein GTPBP10 is involved in mitoribosomal biogenesis.
Nucleic acids research
Lavdovskaia E,Kolander E,Steube E,Mai MM,Urlaub H,Richter-Dennerlein R
Wed Sep 19 00:00:00 UTC 2018
Concerted regulation of mitochondrial and nuclear non-coding RNAs by a dual-targeted RNase Z.
EMBO reports
Siira SJ,Rossetti G,Richman TR,Perks K,Ermer JA,Kuznetsova I,Hughes L,Shearwood AJ,Viola HM,Hool LC,Rackham O,Filipovska A
Mon Oct 01 00:00:00 UTC 2018
Concerted regulation of mitochondrial and nuclear non-coding RNAs by a dual-targeted RNase Z.
EMBO reports
Siira SJ,Rossetti G,Richman TR,Perks K,Ermer JA,Kuznetsova I,Hughes L,Shearwood AJ,Viola HM,Hool LC,Rackham O,Filipovska A
Mon Oct 01 00:00:00 UTC 2018
Biallelic Mutations in MRPS34 Lead to Instability of the Small Mitoribosomal Subunit and Leigh Syndrome.
American journal of human genetics
Lake NJ,Webb BD,Stroud DA,Richman TR,Ruzzenente B,Compton AG,Mountford HS,Pulman J,Zangarelli C,Rio M,Boddaert N,Assouline Z,Sherpa MD,Schadt EE,Houten SM,Byrnes J,McCormick EM,Zolkipli-Cunningham Z,Haude K,Zhang Z,Retterer K,Bai R,Calvo SE,Mootha VK,Christodoulou J,Rötig A,Filipovska A,Cristian I,Falk MJ,Metodiev MD,Thorburn DR
Thu Aug 03 00:00:00 UTC 2017
Biallelic Mutations in MRPS34 Lead to Instability of the Small Mitoribosomal Subunit and Leigh Syndrome.
American journal of human genetics
Lake NJ,Webb BD,Stroud DA,Richman TR,Ruzzenente B,Compton AG,Mountford HS,Pulman J,Zangarelli C,Rio M,Boddaert N,Assouline Z,Sherpa MD,Schadt EE,Houten SM,Byrnes J,McCormick EM,Zolkipli-Cunningham Z,Haude K,Zhang Z,Retterer K,Bai R,Calvo SE,Mootha VK,Christodoulou J,Rötig A,Filipovska A,Cristian I,Falk MJ,Metodiev MD,Thorburn DR
Thu Aug 03 00:00:00 UTC 2017
Adult-onset obesity is triggered by impaired mitochondrial gene expression.
Science advances
Perks KL,Ferreira N,Richman TR,Ermer JA,Kuznetsova I,Shearwood AJ,Lee RG,Viola HM,Johnstone VPA,Matthews V,Hool LC,Rackham O,Filipovska A
Tue Aug 01 00:00:00 UTC 2017
Adult-onset obesity is triggered by impaired mitochondrial gene expression.
Science advances
Perks KL,Ferreira N,Richman TR,Ermer JA,Kuznetsova I,Shearwood AJ,Lee RG,Viola HM,Johnstone VPA,Matthews V,Hool LC,Rackham O,Filipovska A
Tue Aug 01 00:00:00 UTC 2017
Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly.
Cell reports
Rackham O,Busch JD,Matic S,Siira SJ,Kuznetsova I,Atanassov I,Ermer JA,Shearwood AM,Richman TR,Stewart JB,Mourier A,Milenkovic D,Larsson NG,Filipovska A
Tue Aug 16 00:00:00 UTC 2016
Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly.
Cell reports
Rackham O,Busch JD,Matic S,Siira SJ,Kuznetsova I,Atanassov I,Ermer JA,Shearwood AM,Richman TR,Stewart JB,Mourier A,Milenkovic D,Larsson NG,Filipovska A
Tue Aug 16 00:00:00 UTC 2016
Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice.
Nature communications
Richman TR,Spåhr H,Ermer JA,Davies SM,Viola HM,Bates KA,Papadimitriou J,Hool LC,Rodger J,Larsson NG,Rackham O,Filipovska A
Mon Jun 20 00:00:00 UTC 2016
Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice.
Nature communications
Richman TR,Spåhr H,Ermer JA,Davies SM,Viola HM,Bates KA,Papadimitriou J,Hool LC,Rodger J,Larsson NG,Rackham O,Filipovska A
Mon Jun 20 00:00:00 UTC 2016
Mutation in MRPS34 compromises protein synthesis and causes mitochondrial dysfunction.
PLoS genetics
Richman TR,Ermer JA,Davies SM,Perks KL,Viola HM,Shearwood AM,Hool LC,Rackham O,Filipovska A
Sun Mar 01 00:00:00 UTC 2015
Mutation in MRPS34 compromises protein synthesis and causes mitochondrial dysfunction.
PLoS genetics
Richman TR,Ermer JA,Davies SM,Perks KL,Viola HM,Shearwood AM,Hool LC,Rackham O,Filipovska A
Sun Mar 01 00:00:00 UTC 2015