Biblio

Author Title Type [ Year(Asc)]
Filters: Author is Bindoff, Laurence A  [Clear All Filters]
2024
Chen A, Yangzom T, Hong Y, Lundberg BChristian, Sullivan GJohn, Tzoulis C, Bindoff LA, Liang KXiao. Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids. Adv Sci (Weinh). 2024:e2307136.
Hong Y, Zhang Z, Yangzom T, Chen A, Lundberg BChristian, Fang EFei, Siller R, Sullivan GJohn, Zeman J, Tzoulis C, et al. The NAD Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC derived Cortical Organoid of Alpers' Disease. Int J Biol Sci. 2024;20(4):1194-1217.
2023
Marjan S, Yangzom T, Kristiansen CKatrin, Chen A, Bindoff LA, Liang KXiao. Application of Flow Cytometric Analysis for Measuring Multiple Mitochondrial Parameters in 3D Brain Organoids. J Vis Exp. 2023;(198).
Kristiansen CKatrin, Furriol J, Chen A, Sullivan GJohn, Bindoff LA, Liang KXiao. Deoxyribonucleoside treatment rescues EtBr-induced mtDNA depletion in iPSC-derived neural stem cells with POLG mutations. FASEB J. 2023;37(9):e23139.
Hong Y, Kristiansen CKatrin, Chen A, Nido GSanchez, Høyland LElise, Ziegler M, Sullivan GJohn, Bindoff LA, Liang KXiao. POLG genotype influences degree of mitochondrial dysfunction in iPSC derived neural progenitors, but not the parent iPSC or derived glia. Exp Neurol. 2023:114429.
2022
Chen A, Kristiansen CKatrin, Høyland LElise, Ziegler M, Wang J, Sullivan GJohn, Li X, Bindoff LA, Liang KXiao. mutations lead to abnormal mitochondrial remodeling during neural differentiation of human pluripotent stem cells via SIRT3/AMPK pathway inhibition. Cell Cycle. 2022:1-16.
2020
Liang KXiao, Vatne GHelén, Kristiansen CKatrin, Ievglevskyi O, Kondratskaya E, Glover JC, Chen A, Sullivan GJohn, Bindoff LA. N-acetylcysteine amide ameliorates mitochondrial dysfunction and reduces oxidative stress in hiPSC-derived dopaminergic neurons with POLG mutation. Exp Neurol. 2020:113536.
Hikmat O, Naess K, Engvall M, Klingenberg C, Rasmussen M, Tallaksen CMe, Brodtkorb E, Ostergaard E, de Coo IFM, Pias-Peleteiro L, et al. Simplifying the clinical classification of polymerase gamma (POLG) disease based on age of onset; studies using a cohort of 155 cases. J Inherit Metab Dis. 2020.
Varhaug KN, Nido GS, de Coo I, Isohanni P, Suomalainen A, Tzoulis C, Knappskog P, Bindoff LA. Using urine to diagnose large-scale mtDNA deletions in adult patients. Ann Clin Transl Neurol. 2020.
2014
Yarham JW, Lamichhane TN, Pyle A, Mattijssen S, Baruffini E, Bruni F, Donnini C, Vassilev A, He L, Blakely EL, et al. Defective i6A37 Modification of Mitochondrial and Cytosolic tRNAs Results from Pathogenic Mutations in TRIT1 and Its Substrate tRNA. PLoS Genet. 2014;10(6):e1004424.