Guro Helén Vatne

• Grødem, Sverre; Nymoen, Ingeborg; Vatne, Guro Helén; Rogge, Frederik Sebastian; Björnsdottir, Valgerdur & Lensjø, Kristian [Show all 7 contributors for this article] (2023). An updated suite of viral vectors for in vivo calcium imaging using intracerebral and retro-orbital injections in male mice. Nature Communications. ISSN 2041-1723. 14(1), p. 608–1. doi: 10.1038/s41467-023-36324-3. Full text in Research Archive
• Liang, Xiao; Vatne, Guro Helén; Kristiansen, Cecilie Katrin; Ievglevskyi, Oleksandr; Kondratskaya, Elena & Glover, Joel [Show all 9 contributors for this article] (2020). N-acetylcysteine amide ameliorates mitochondrial dysfunction and reduces oxidative stress in hiPSC-derived dopaminergic neurons with POLG mutation. Experimental Neurology. ISSN 0014-4886. 337. doi: 10.1016/j.expneurol.2020.113536.
• Liang, Xiao; Kristiansen, Cecilie Katrin; Mostafavi, Sepideh; Vatne, Guro Helén; Zantingh, Gina Alien & Kianian, Atefeh [Show all 17 contributors for this article] (2020). Disease-specific phenotypes in iPSC-derived neural stem cells with POLG mutations. EMBO Molecular Medicine. ISSN 1757-4676. 12(10), p. 1–26. doi: 10.15252/emmm.202012146. Full text in Research Archive Show summary

  Mutations in POLG disrupt mtDNA replication and cause devas- tating diseases often with neurological phenotypes. Defining disease mechanisms has been hampered by limited access to human tissues, particularly neurons. Using patient cells carrying POLG mutations, we generated iPSCs and then neural stem cells. These neural precursors manifested a phenotype that faithfully replicated the molecular and biochemical changes found in patient post-mortem brain tissue. We confirmed the same loss of mtDNA and complex I in dopaminergic neurons generated from the same stem cells. POLG-driven mitochondrial dysfunc- tion led to neuronal ROS overproduction and increased cellular senescence. Loss of complex I was associated with disturbed NAD+ metabolism with increased UCP2 expression and reduced phosphorylated SirT1. In cells with compound heterozygous POLG mutations, we also found activated mitophagy via the BNIP3 pathway. Our studies are the first that show it is possible to recapitulate the neuronal molecular and biochemical defects associated with POLG mutation in a human stem cell model. Further, our data provide insight into how mitochondrial dysfunction and mtDNA alterations influence cellular fate deter- mining processes.

• Liang, Xiao; Kristiansen, Cecilie Katrin; Vatne, Guro Helén; Hong, Yu & Bindoff, Laurence (2020). Patient-specific neural progenitor cells derived from induced pluripotent stem cells offer a promise of good models for mitochondrial disease. Cell and Tissue Research. ISSN 0302-766X. 380(1), p. 15–30. doi: 10.1007/s00441-019-03164-x.
• Raasakka, Arne; Ruskamo, Salla; Barker, Robert; Krokengen, Oda Caspara; Vatne, Guro Helén & Kristiansen, Cecilie Katrin [Show all 13 contributors for this article] (2019). Neuropathy-related mutations alter the membrane binding properties of the human myelin protein P0 cytoplasmic tail. PLOS ONE. ISSN 1932-6203. 14:e0216833(6), p. 1–24. doi: 10.1371/journal.pone.0216833. Full text in Research Archive
• Ruskamo, Salla; Nieminen, Tuomo; Kristiansen, Cecilie Katrin; Vatne, Guro Helén; Baumann, Anne & Hallin, Erik Ingmar [Show all 11 contributors for this article] (2017). Molecular mechanisms of Charcot-Marie-Tooth neuropathy linked to mutations in human myelin protein P2. Scientific Reports. ISSN 2045-2322. 7:6510, p. 1–13. doi: 10.1038/s41598-017-06781-0. Full text in Research Archive

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• Ruskamo, Salla; Nieminen, Tuomo; Kristiansen, Cecilie Katrin; Vatne, Guro Helén; Baumann, Anne & Hallin, Erik Ingmar [Show all 11 contributors for this article] (2018). Erratum: Publisher Correction: Molecular mechanisms of Charcot-Marie-Tooth neuropathy linked to mutations in human myelin protein P2. Scientific Reports. ISSN 2045-2322. 8. doi: 10.1038/s41598-017-18751-7.

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