Laurence A. Bindoff

Haukeland University Hospital — Norway

Biochemistry, Genetics and Molecular Biology · Life Sciences

62h-index13.9kcitations293works6.52yr avg
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Research Topics

Mitochondrial Function and Pathology(150), Metabolism and Genetic Disorders(97), Genetic Neurodegenerative Diseases(37), ATP Synthase and ATPases Research(32), Muscle Physiology and Disorders(20)

Publications293 total

Biomarking MELAS with neurofilament light chain and circulating cell free mitochondrial DNA
Molecular Genetics and Metabolism·2026Open Access
Epidemiology and natural history of POLG disease in Norway: a nationwide cohort study
Annals of Clinical and Translational Neurology·2024· 5 citedOpen Access
Status epilepticus in POLG disease: a large multinational study
Journal of Neurology·2024· 14 citedOpen Access
Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids
Advanced Science·2024· 7 citedOpen Access
Utility and limitations of EEG in the diagnosis and management of ALDH7A1-related pyridoxine-dependent epilepsy. A retrospective observational study
Frontiers in Neurology·2024· 3 citedOpen Access
Elevated cholesterol in <i>ATAD3</i> mutants is a compensatory mechanism that leads to membrane cholesterol aggregation
Brain·2024· 7 citedOpen Access
Activation of Neurotoxic Astrocytes Due to Mitochondrial Dysfunction Triggered by <i>POLG</i> Mutation
International Journal of Biological Sciences·2024· 11 citedOpen Access
The NAD <sup>+</sup> Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC derived Cortical Organoid of Alpers' Disease
International Journal of Biological Sciences·2024· 5 citedOpen Access
Hallmark molecular and pathological features of POLG disease are recapitulated in cerebral organoids
2023· 2 citedOpen Access
Mit.OnOff: A Science Communication Project for Public Awareness about Mitochondrial Cytopathies
Endocrine Metabolic & Immune Disorders - Drug Targets·2023
Deoxyribonucleoside treatment rescues <scp>EtBr</scp> ‐induced <scp>mtDNA</scp> depletion in <scp>iPSC</scp> ‐derived neural stem cells with <scp>POLG</scp> mutations
The FASEB Journal·2023· 5 citedOpen Access
Application of Flow Cytometric Analysis for Measuring Multiple Mitochondrial Parameters in 3D Brain Organoids
Journal of Visualized Experiments·2023· 1 cited
PPAR-gamma agonist pioglitazone recovers mitochondrial quality control in fibroblasts from PITRM1-deficient patients
Frontiers in Pharmacology·2023· 8 citedOpen Access
The NAD <sup>+</sup> Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC derived Cortical Organoid of Alpers’ Disease
2023· 1 citedOpen Access
POLG genotype influences degree of mitochondrial dysfunction in iPSC derived neural progenitors, but not the parent iPSC or derived glia
Experimental Neurology·2023· 6 citedOpen Access
Corrigendum to “The spectrum of pyridoxine dependent epilepsy across the age span: A nationwide retrospective observational study” [Epilepsy Res. 190 (2023) 107099]
Epilepsy Research·2023· 1 cited
POLG genotype influences degree of mitochondrial dysfunction in iPSC derived neural progenitors, but not the parent iPSC or derived glia
2023· 2 citedOpen Access
The spectrum of pyridoxine dependent epilepsy across the age span: A nationwide retrospective observational study
Epilepsy Research·2023· 7 citedOpen Access
Epidemiology and natural history in 101 subjects with FKRP-related limb-girdle muscular dystrophy R9. The Norwegian LGMDR9 cohort study (2020)
Neuromuscular Disorders·2022· 14 citedOpen Access
Musculoskeletal Chronic Graft versus Host Disease—A Rare Complication to Allogeneic Hematopoietic Stem Cell Transplant: A Case-Based Report and Review of the Literature
Current Oncology·2022· 5 citedOpen Access
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Frequent Co-authors

Douglass M. Turnbull(23), Patrick F. Chinnery(10), Massimo Zeviani(8), Robert McFarland(7), Charalampos Tzoulis(7), Rita Horváth(6), Joanna Poulton(6), Anu Suomalainen(5), Robert W. Taylor(5), Thomas Klopstock(5), Gráinne S. Gorman(4), Michio Hirano(4), Neil Howell(4), Mark A. Birch‐Machin(4), Michelangelo Mancuso(4), Margaret Jackson(3), Philip G. Griffiths(3), Valério Carelli(3), Anna H. Hakonen(3), Jan Smeıtınk(3)