Browsing Bergen Open Research Archive by Author "Sullivan, Gareth John"
Now showing items 1-9 of 9
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Activation of Neurotoxic Astrocytes Due to Mitochondrial Dysfunction Triggered by POLG Mutation
Liang, Xiao; Chen, Anbin; Kianian, Atefeh; Kristiansen, Cecilie Katrin; Tsering Yangzom, Tsering Yangzom; Furriol Palmer, Jessica; Høyland, Lena Elise; Ziegler, Mathias; Kråkenes, Torbjørn; Tzoulis, Charalampos; Fang, Fei; Sullivan, Gareth John; Bindoff, Laurence Albert (Journal article; Peer reviewed, 2024)Mitochondrial diseases are associated with neuronal death and mtDNA depletion. Astrocytes respond to injury or stimuli and damage to the central nervous system. Neurodegeneration can cause astrocytes to activate and acquire ... -
Comparing the mitochondrial signatures in ESCs and iPSCs and their neural derivations
Kristiansen, Cecilie Katrin; Chen, Anbin; Høyland, Lena Elise; Ziegler, Mathias; Sullivan, Gareth John; Bindoff, Laurence Albert; Liang, Kristina Xiao (Journal article; Peer reviewed, 2022)Embryonic stem cells (ESCs) and induced pluripotent stem cells (iPSCs) have distinct origins: ESCs are derived from pre-implanted embryos while iPSCs are reprogrammed somatic cells. Both have their own characteristics and ... -
Deoxyribonucleoside treatment rescues EtBr-induced mtDNA depletion in iPSC-derived neural stem cells with POLG mutations
Kristiansen, Cecilie Katrin; Furriol, Jessica; Chen, Anbin; Sullivan, Gareth John; Bindoff, Laurence Albert; Liang, Xiao (Journal article; Peer reviewed, 2023)Mutations in POLG, the gene encoding the catalytic subunit of the mitochondrial DNA (mtDNA) polymerase gamma (Pol-γ), lead to diseases driven by defective mtDNA maintenance. Despite being the most prevalent cause of ... -
Distinct Mitochondrial Remodeling During Mesoderm Differentiation in a Human-Based Stem Cell Model
Mostafavi, Sepideh; Balafkan, Novin; Pettersen, Ina Katrine Nitschke; Nido, Gonzalo Sanchez; Siller, Richard; Tzoulis, Charalampos; Sullivan, Gareth John; Bindoff, Laurence Albert (Journal article; Peer reviewed, 2021-10-14)Given the considerable interest in using stem cells for modeling and treating disease, it is essential to understand what regulates self-renewal and differentiation. Remodeling of mitochondria and metabolism, with the shift ... -
Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids
Chen, Anbin; Yangzom, Tsering; Hong, Yu; Lundberg, Bjørn Christian; Sullivan, Gareth John; Tzoulis, Charalampos; Bindoff, Laurence Albert; Liang, Xiao (Journal article; Peer reviewed, 2024)In this research, a 3D brain organoid model is developed to study POLG-related encephalopathy, a mitochondrial disease stemming from POLG mutations. Induced pluripotent stem cells (iPSCs) derived from patients with these ... -
The NAD+ Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC derived Cortical Organoid of Alpers' Disease
Hong, Yu; Zhang, Zhuoyuan; Yangzom, Tsering; Chen, Anbin; Lundberg, Bjørn Christian; Fang, Fei; Siller, Richard; Sullivan, Gareth John; Zeman, Jiri; Tzoulis, Charalampos; Bindoff, Laurence Albert; Liang, Xiao (Journal article; Peer reviewed, 2024)Alpers' syndrome is an early-onset neurodegenerative disorder usually caused by biallelic pathogenic variants in the gene encoding the catalytic subunit of polymerase-gamma (POLG), which is essential for mitochondrial DNA ... -
Nicotinamide Riboside and Metformin Ameliorate Mitophagy Defect in Induced Pluripotent Stem Cell-Derived Astrocytes With POLG Mutations
Chen, Anbin; Kristiansen, Cecilie Katrin; Hong, Yu; Kianian, Atefeh; Fang, Evandro Fei; Sullivan, Gareth John; Wang, Jian; Li, X.; Bindoff, Laurence Albert; Liang, Xiao (Journal article; Peer reviewed, 2021)Mitophagy specifically recognizes and removes damaged or superfluous mitochondria to maintain mitochondrial homeostasis and proper neuronal function. Defective mitophagy and the resulting accumulation of damaged mitochondria ... -
POLG genotype influences degree of mitochondrial dysfunction in iPSC derived neural progenitors, but not the parent iPSC or derived glia
Hong, Yu; Kristiansen, Cecilie Katrin; Chen, Anbin; Sanchez Nido, Gonzalo; Høyland, Lena Elise; Ziegler, Mathias; Sullivan, Gareth John; Bindoff, Laurence Albert; Liang, Xiao (Journal article; Peer reviewed, 2023)Diseases caused by POLG mutations are the most common form of mitochondrial diseases and associated with phenotypes of varying severity. Clinical studies have shown that patients with compound heterozygous POLG mutations ... -
POLG mutations lead to abnormal mitochondrial remodeling during neural differentiation of human pluripotent stem cells via SIRT3/AMPK pathway inhibition
Chen, Anbin; Kristiansen, Cecilie Katrin; Høyland, Lena Elise; Ziegler, Mathias; Wang, Jian; Sullivan, Gareth John; Li, Xingang; Bindoff, Laurence Albert; Liang, Xiao (Journal article; Peer reviewed, 2022)We showed previously that POLG mutations cause major changes in mitochondrial function, including loss of mitochondrial respiratory chain (MRC) complex I, mitochondrial DNA (mtDNA) depletion and an abnormal NAD+/NADH ratio ...