[HTML][HTML] Human pluripotent-stem-cell-derived organoids for drug discovery and evaluation

JJ Vandana, C Manrique, LA Lacko, S Chen - Cell Stem Cell, 2023 - cell.com
Human pluripotent stem cells (hPSCs) and three-dimensional organoids have ushered in a
new era for disease modeling and drug discovery. Over the past decade, significant …

[HTML][HTML] Regulatory roles of mitochondria and metabolism in neurogenesis

R Iwata, P Vanderhaeghen - Current opinion in neurobiology, 2021 - Elsevier
Neural stem cells (NSCs) undergo massive molecular and cellular changes during neuronal
differentiation. These include mitochondria and metabolism remodelling, which were …

[HTML][HTML] OxPhos defects cause hypermetabolism and reduce lifespan in cells and in patients with mitochondrial diseases

G Sturm, KR Karan, AS Monzel, B Santhanam… - Communications …, 2023 - nature.com
Patients with primary mitochondrial oxidative phosphorylation (OxPhos) defects present with
fatigue and multi-system disorders, are often lean, and die prematurely, but the mechanistic …

[HTML][HTML] Mitochondrial protein dysfunction in pathogenesis of neurological diseases

L Wang, Z Yang, X He, S Pu, C Yang, Q Wu… - Frontiers in Molecular …, 2022 - frontiersin.org
Mitochondria are essential organelles for neuronal function and cell survival. Besides the
well-known bioenergetics, additional mitochondrial roles in calcium signaling, lipid …

[HTML][HTML] Epilepsy in mitochondrial diseases—current state of knowledge on aetiology and treatment

D Wesół-Kucharska, D Rokicki, A Jezela-Stanek - Children, 2021 - mdpi.com
Mitochondrial diseases are a heterogeneous group of diseases resulting from energy deficit
and reduced adenosine triphosphate (ATP) production due to impaired oxidative …

[HTML][HTML] Gene therapy for mitochondrial diseases: Current status and future perspective

A Di Donfrancesco, G Massaro, I Di Meo, V Tiranti… - Pharmaceutics, 2022 - mdpi.com
Mitochondrial diseases (MDs) are a group of severe genetic disorders caused by mutations
in the nuclear or mitochondrial genome encoding proteins involved in the oxidative …

[HTML][HTML] Direct neuronal reprogramming of NDUFS4 patient cells identifies the unfolded protein response as a novel general reprogramming hurdle

G Sonsalla, AB Malpartida, T Riedemann, M Gusic… - Neuron, 2024 - cell.com
Mitochondria account for essential cellular pathways, from ATP production to nucleotide
metabolism, and their deficits lead to neurological disorders and contribute to the onset of …

Mitochondria in neurogenesis: Implications for mitochondrial diseases

D Brunetti, W Dykstra, S Le, A Zink, A Prigione - Stem Cells, 2021 - academic.oup.com
Mitochondria are organelles with recognized key roles in cellular homeostasis, including
bioenergetics, redox, calcium signaling, and cell death. Mitochondria are essential for …

[HTML][HTML] Anti-neuronal antibodies against brainstem antigens are associated with COVID-19

G Lucchese, A Vogelgesang, F Boesl, D Raafat… - …, 2022 - thelancet.com
Summary Background Understanding how SARS-CoV-2 affects respiratory centres in the
brainstem may help to preclude assisted ventilation for patients in intensive care setting …

Modeling developmental brain diseases using human pluripotent stem cells-derived brain organoids–progress and perspective

A Bhattacharya, WWY Choi, J Muffat, Y Li - Journal of Molecular Biology, 2022 - Elsevier
Developmental brain diseases encompass a group of conditions resulting from genetic or
environmental perturbations during early development. Despite the increased research …