Product References
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
A variant in MRPS14 (uS14m) causes perinatal hypertrophic cardiomyopathy with neonatal lactic acidosis, growth retardation, dysmorphic features and neurological involvement.
Human molecular genetics
Jackson CB,Huemer M,Bolognini R,Martin F,Szinnai G,Donner BC,Richter U,Battersby BJ,Nuoffer JM,Suomalainen A,Schaller A
Fri Feb 15 00:00:00 UTC 2019
A variant in MRPS14 (uS14m) causes perinatal hypertrophic cardiomyopathy with neonatal lactic acidosis, growth retardation, dysmorphic features and neurological involvement.
Human molecular genetics
Jackson CB,Huemer M,Bolognini R,Martin F,Szinnai G,Donner BC,Richter U,Battersby BJ,Nuoffer JM,Suomalainen A,Schaller A
Fri Feb 15 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
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
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