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
Integrative genetic analysis identifies FLVCR1 as a plasma-membrane choline transporter in mammals.
Cell metabolism
Kenny TC,Khan A,Son Y,Yue L,Heissel S,Sharma A,Pasolli HA,Liu Y,Gamazon ER,Alwaseem H,Hite RK,Birsoy K
Tue Jun 06 00:00:00 UTC 2023
Sucrose Gradient Analysis of Human Mitochondrial Ribosomes and RNA.
Methods in molecular biology (Clifton, N.J.)
Ng KY,Battersby BJ
Mon May 15 00:00:00 UTC 2023
circMRPS35 promotes malignant progression and cisplatin resistance in hepatocellular carcinoma.
Molecular therapy : the journal of the American Society of Gene Therapy
Li P,Song R,Yin F,Liu M,Liu H,Ma S,Jia X,Lu X,Zhong Y,Yu L,Li X,Li X
Wed Jan 05 00:00:00 UTC 2022
circMRPS35 promotes malignant progression and cisplatin resistance in hepatocellular carcinoma.
Molecular therapy : the journal of the American Society of Gene Therapy
Li P,Song R,Yin F,Liu M,Liu H,Ma S,Jia X,Lu X,Zhong Y,Yu L,Li X,Li X
Wed Jan 05 00:00:00 UTC 2022
Nonstop mRNAs generate a ground state of mitochondrial gene expression noise.
Science advances
Ng KY,Lutfullahoglu Bal G,Richter U,Safronov O,Paulin L,Dunn CD,Paavilainen VO,Richer J,Newman WG,Taylor RW,Battersby BJ
Wed Nov 16 00:00:00 UTC 2022
Dysfunctional mitochondrial translation and combined oxidative phosphorylation deficiency in a mouse model of hepatoencephalopathy due to Gfm1 mutations.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology
Molina-Berenguer M,Vila-Julià F,Pérez-Ramos S,Salcedo-Allende MT,Cámara Y,Torres-Torronteras J,Martí R
Sat Jan 01 00:00:00 UTC 2022
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
Linear Density Sucrose Gradients to Study Mitoribosomal Biogenesis in Tissue-Specific Knockout Mice.
Methods in molecular biology (Clifton, N.J.)
Ruzzenente B,Metodiev MD
Thu Apr 01 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
TRMT2B is responsible for both tRNA and rRNA m5U-methylation in human mitochondria.
RNA biology
Powell CA,Minczuk M
Wed Apr 01 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
RNase H1 Regulates Mitochondrial Transcription and Translation via the Degradation of 7S RNA.
Frontiers in genetics
Reyes A,Rusecka J,Tońska K,Zeviani M
Thu Feb 09 00:00:00 UTC 2023
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
Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals.
eLife
Kühl I,Miranda M,Atanassov I,Kuznetsova I,Hinze Y,Mourier A,Filipovska A,Larsson NG
Tue Nov 14 00:00:00 UTC 2017
Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals.
eLife
Kühl I,Miranda M,Atanassov I,Kuznetsova I,Hinze Y,Mourier A,Filipovska A,Larsson NG
Tue Nov 14 00:00:00 UTC 2017
CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels.
The EMBO journal
Szczepanowska K,Maiti P,Kukat A,Hofsetz E,Nolte H,Senft K,Becker C,Ruzzenente B,Hornig-Do HT,Wibom R,Wiesner RJ,Krüger M,Trifunovic A
Thu Dec 01 00:00:00 UTC 2016
CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels.
The EMBO journal
Szczepanowska K,Maiti P,Kukat A,Hofsetz E,Nolte H,Senft K,Becker C,Ruzzenente B,Hornig-Do HT,Wibom R,Wiesner RJ,Krüger M,Trifunovic A
Thu Dec 01 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
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
SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation.
PLoS genetics
Lagouge M,Mourier A,Lee HJ,Spåhr H,Wai T,Kukat C,Silva Ramos E,Motori E,Busch JD,Siira S,Kremmer E,Filipovska A,Larsson NG
Sat Aug 01 00:00:00 UTC 2015
SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation.
PLoS genetics
Lagouge M,Mourier A,Lee HJ,Spåhr H,Wai T,Kukat C,Silva Ramos E,Motori E,Busch JD,Siira S,Kremmer E,Filipovska A,Larsson NG
Sat Aug 01 00:00:00 UTC 2015
Tissue-specific loss of DARS2 activates stress responses independently of respiratory chain deficiency in the heart.
Cell metabolism
Dogan SA,Pujol C,Maiti P,Kukat A,Wang S,Hermans S,Senft K,Wibom R,Rugarli EI,Trifunovic A
Tue Mar 04 00:00:00 UTC 2014
AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival.
The Journal of clinical investigation
Almajan ER,Richter R,Paeger L,Martinelli P,Barth E,Decker T,Larsson NG,Kloppenburg P,Langer T,Rugarli EI
Thu Nov 01 00:00:00 UTC 2012
AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival.
The Journal of clinical investigation
Almajan ER,Richter R,Paeger L,Martinelli P,Barth E,Decker T,Larsson NG,Kloppenburg P,Langer T,Rugarli EI
Thu Nov 01 00:00:00 UTC 2012