J
Joni Nikkanen
Researcher at University of Helsinki
Publications - 11
Citations - 871
Joni Nikkanen is an academic researcher from University of Helsinki. The author has contributed to research in topics: Mitochondrial myopathy & Mitochondrial disease. The author has an hindex of 6, co-authored 8 publications receiving 556 citations. Previous affiliations of Joni Nikkanen include University of California, San Francisco.
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Journal ArticleDOI
mTORC1 Regulates Mitochondrial Integrated Stress Response and Mitochondrial Myopathy Progression
Nahid Akhtar Khan,Joni Nikkanen,Shuichi Yatsuga,Shuichi Yatsuga,Christopher B. Jackson,Liya Wang,Swagat Pradhan,Riikka Kivelä,Alberto Pessia,Vidya Velagapudi,Anu Suomalainen +10 more
TL;DR: The evidence suggests that chronic upregulation of anabolic pathways contributes to MM progression, long-term induction of ISRmt is not protective for muscle, and rapamycin treatment trials should be considered for adult-type MM with raised FGF21.
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Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism
Joni Nikkanen,Saara Forsström,Liliya Euro,Ilse Paetau,Rebecca A. Kohnz,Liya Wang,Dmitri Chilov,Jenni Viinamäki,Anne Roivainen,Päivi Marjamäki,Heidi Liljenbäck,Sofia Ahola,Jana Buzkova,Mügen Terzioglu,Nahid Akhtar Khan,Sini Pirnes-Karhu,Anders Paetau,Tuula Lönnqvist,Antti Sajantila,Pirjo Isohanni,Henna Tyynismaa,Daniel K. Nomura,Brendan J. Battersby,Vidya Velagapudi,Christopher Carroll,Anu Suomalainen +25 more
TL;DR: It is reported that mtDNA replication disorders caused by TWINKLE mutations-mitochondrial myopathy (MM) and infantile onset spinocerebellar ataxia (IOSCA)-remodel cellular dNTP pools in mice are reported and targets for metabolic therapy are offered.
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Energetic Trade-Offs and Hypometabolic States Promote Disease Tolerance.
Kirthana Ganeshan,Joni Nikkanen,Kevin Man,Yew Ann Leong,Yew Ann Leong,Yoshitaka Sogawa,J. Alan Maschek,Tyler Van Ry,D. Nyasha Chagwedera,James E. Cox,Ajay Chawla +10 more
TL;DR: It is reported here that activation of immunity causes an energetic trade-off with the homeothermy (the stable maintenance of core temperature), resulting in hypometabolism and hypothermia, suggesting that energy-conserving hypometabolic states, such as dormancy, might have evolved as a mechanism of tissue tolerance.
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Fibroblast Growth Factor 21 Drives Dynamics of Local and Systemic Stress Responses in Mitochondrial Myopathy with mtDNA Deletions
Saara Forsström,Christopher B. Jackson,Christopher Carroll,Christopher Carroll,Mervi Kuronen,Eija Pirinen,Swagat Pradhan,Anastasiia Marmyleva,Mari Auranen,Iida-Marja Kleine,Nahid Akhtar Khan,Anne Roivainen,Päivi Marjamäki,Heidi Liljenbäck,Liya Wang,Brendan J. Battersby,Uwe Richter,Vidya Velagapudi,Joni Nikkanen,Liliya Euro,Anu Suomalainen,Anu Suomalainen +21 more
TL;DR: The evidence indicates that FGF21 is a local and systemic messenger of mtDNA stress in mice and humans with mitochondrial disease, and drives weight loss and glucose preference, and modifies metabolism and respiratory chain deficiency in a specific hippocampal brain region.
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Metabolomes of mitochondrial diseases and inclusion body myositis patients: treatment targets and biomarkers.
Jana Buzkova,Joni Nikkanen,Sofia Ahola,Anna H. Hakonen,Ksenia Sevastianova,Ksenia Sevastianova,Topi Hovinen,Hannele Yki-Järvinen,Hannele Yki-Järvinen,Kirsi H. Pietiläinen,Kirsi H. Pietiläinen,Tuula Lönnqvist,Vidya Velagapudi,Christopher Carroll,Christopher Carroll,Anu Suomalainen +15 more
TL;DR: The omics approach identified pathways currently used to treat NMDs and mitochondrial stroke‐like episodes and proposes nicotinamide riboside in MDs and IBM, and creatine in IOSCA and IBM as novel treatment targets.