Product References
Restoration of mitochondrial function through activation of hypomodified tRNAs with pathogenic mutations associated with mitochondrial diseases.
Nucleic acids research
Tomoda E,Nagao A,Shirai Y,Asano K,Suzuki T,Battersby BJ,Suzuki T
Fri Aug 11 00:00:00 UTC 2023
The human tRNA taurine modification enzyme GTPBP3 is an active GTPase linked to mitochondrial diseases.
Nucleic acids research
Peng GX,Zhang Y,Wang QQ,Li QR,Xu H,Wang ED,Zhou XL
Thu Mar 18 00:00:00 UTC 2021
The MELAS mutation m.3243A>G alters the expression of mitochondrial tRNA fragments.
Biochimica et biophysica acta. Molecular cell research
Meseguer S,Navarro-González C,Panadero J,Villarroya M,Boutoual R,Sánchez-Alcázar JA,Armengod ME
Sun Sep 01 00:00:00 UTC 2019
RNA modification landscape of the human mitochondrial tRNALys regulates protein synthesis.
Nature communications
Richter U,Evans ME,Clark WC,Marttinen P,Shoubridge EA,Suomalainen A,Wredenberg A,Wedell A,Pan T,Battersby BJ
Thu Sep 27 00:00:00 UTC 2018
RNA modification landscape of the human mitochondrial tRNALys regulates protein synthesis.
Nature communications
Richter U,Evans ME,Clark WC,Marttinen P,Shoubridge EA,Suomalainen A,Wredenberg A,Wedell A,Pan T,Battersby BJ
Thu Sep 27 00:00:00 UTC 2018
Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARγ-UCP2-AMPK axis.
Scientific reports
Boutoual R,Meseguer S,Villarroya M,Martín-Hernández E,Errami M,Martín MA,Casado M,Armengod ME
Thu Jan 18 00:00:00 UTC 2018
Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARγ-UCP2-AMPK axis.
Scientific reports
Boutoual R,Meseguer S,Villarroya M,Martín-Hernández E,Errami M,Martín MA,Casado M,Armengod ME
Thu Jan 18 00:00:00 UTC 2018
microRNA-mediated differential expression of TRMU, GTPBP3 and MTO1 in cell models of mitochondrial-DNA diseases.
Scientific reports
Meseguer S,Boix O,Navarro-González C,Villarroya M,Boutoual R,Emperador S,García-Arumí E,Montoya J,Armengod ME
Mon Jul 24 00:00:00 UTC 2017
Severe respiratory complex III defect prevents liver adaptation to prolonged fasting.
Journal of hepatology
Kremer LS,L'hermitte-Stead C,Lesimple P,Gilleron M,Filaut S,Jardel C,Haack TB,Strom TM,Meitinger T,Azzouz H,Tebib N,Ogier de Baulny H,Touati G,Prokisch H,Lombès A
Mon Aug 01 00:00:00 UTC 2016
Severe respiratory complex III defect prevents liver adaptation to prolonged fasting.
Journal of hepatology
Kremer LS,L'hermitte-Stead C,Lesimple P,Gilleron M,Filaut S,Jardel C,Haack TB,Strom TM,Meitinger T,Azzouz H,Tebib N,Ogier de Baulny H,Touati G,Prokisch H,Lombès A
Mon Aug 01 00:00:00 UTC 2016
The ROS-sensitive microRNA-9/9* controls the expression of mitochondrial tRNA-modifying enzymes and is involved in the molecular mechanism of MELAS syndrome.
Human molecular genetics
Meseguer S,Martínez-Zamora A,García-Arumí E,Andreu AL,Armengod ME
Thu Jan 01 00:00:00 UTC 2015
The ROS-sensitive microRNA-9/9* controls the expression of mitochondrial tRNA-modifying enzymes and is involved in the molecular mechanism of MELAS syndrome.
Human molecular genetics
Meseguer S,Martínez-Zamora A,García-Arumí E,Andreu AL,Armengod ME
Thu Jan 01 00:00:00 UTC 2015
Altered 2-thiouridylation impairs mitochondrial translation in reversible infantile respiratory chain deficiency.
Human molecular genetics
Boczonadi V,Smith PM,Pyle A,Gomez-Duran A,Schara U,Tulinius M,Chinnery PF,Horvath R
Fri Nov 15 00:00:00 UTC 2013
Altered 2-thiouridylation impairs mitochondrial translation in reversible infantile respiratory chain deficiency.
Human molecular genetics
Boczonadi V,Smith PM,Pyle A,Gomez-Duran A,Schara U,Tulinius M,Chinnery PF,Horvath R
Fri Nov 15 00:00:00 UTC 2013
Mutations of the mitochondrial-tRNA modifier MTO1 cause hypertrophic cardiomyopathy and lactic acidosis.
American journal of human genetics
Ghezzi D,Baruffini E,Haack TB,Invernizzi F,Melchionda L,Dallabona C,Strom TM,Parini R,Burlina AB,Meitinger T,Prokisch H,Ferrero I,Zeviani M
Fri Jun 08 00:00:00 UTC 2012
Mutations of the mitochondrial-tRNA modifier MTO1 cause hypertrophic cardiomyopathy and lactic acidosis.
American journal of human genetics
Ghezzi D,Baruffini E,Haack TB,Invernizzi F,Melchionda L,Dallabona C,Strom TM,Parini R,Burlina AB,Meitinger T,Prokisch H,Ferrero I,Zeviani M
Fri Jun 08 00:00:00 UTC 2012