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Liu Z, Zeinalzadeh Z, Huang T, Han Y, Peng L, Wang D, Zhou Z, Ousmane D, Wang J
Translational oncology 2024 Apr;42:101896
A STAT3 protein complex required for mitochondrial mRNA stability and cancer.
Fernando CD, Jayasekara WSN, Inampudi C, Kohonen-Corish MRJ, Cooper WA, Beilharz TH, Josephs TM, Garama DJ, Gough DJ
Cell reports 2023 Sep 26;42(9):113033
Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan.
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
Aging cell 2021 Jul;20(7):e13408
A variant in MRPS14 (uS14m) causes perinatal hypertrophic cardiomyopathy with neonatal lactic acidosis, growth retardation, dysmorphic features and neurological involvement.
Jackson CB, Huemer M, Bolognini R, Martin F, Szinnai G, Donner BC, Richter U, Battersby BJ, Nuoffer JM, Suomalainen A, Schaller A
Human molecular genetics 2019 Feb 15;28(4):639-649
Fidelity of translation initiation is required for coordinated respiratory complex assembly.
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
Science advances 2019 Dec;5(12):eaay2118
Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation.
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
The EMBO journal 2019 Dec 16;38(24):e102155
Concerted regulation of mitochondrial and nuclear non-coding RNAs by a dual-targeted RNase Z.
Siira SJ, Rossetti G, Richman TR, Perks K, Ermer JA, Kuznetsova I, Hughes L, Shearwood AJ, Viola HM, Hool LC, Rackham O, Filipovska A
EMBO reports 2018 Oct;19(10)
Adult-onset obesity is triggered by impaired mitochondrial gene expression.
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
Science advances 2017 Aug;3(8):e1700677
Biallelic Mutations in MRPS34 Lead to Instability of the Small Mitoribosomal Subunit and Leigh Syndrome.
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
American journal of human genetics 2017 Aug 3;101(2):239-254
Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly.
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
Cell reports 2016 Aug 16;16(7):1874-90