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 …

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] Linking mitochondria metabolism, developmental timing, and human brain evolution

P Casimir, R Iwata, P Vanderhaeghen - Current opinion in genetics & …, 2024 - Elsevier
Highlights•The human cerebral cortex is characterized by a protracted post-natal
development (neoteny) compared with other species.•The species-specific rate of cortical …

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 …

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 …

Modeling mitochondrial DNA diseases: from base editing to pluripotent stem‐cell‐derived organoids

I Tolle, V Tiranti, A Prigione - EMBO reports, 2023 - embopress.org
Mitochondrial DNA (mtDNA) diseases are multi‐systemic disorders caused by mutations
affecting a fraction or the entirety of mtDNA copies. Currently, there are no approved …

Advanced cellular models for rare disease study: exploring neural, muscle and skeletal organoids

C Bombieri, A Corsi, E Trabetti, A Ruggiero… - International Journal of …, 2024 - mdpi.com
Organoids are self-organized, three-dimensional structures derived from stem cells that can
mimic the structure and physiology of human organs. Patient-specific induced pluripotent …

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 …

The energetics of cellular life transitions

AS Monzel, M Levin, M Picard - Life metabolism, 2024 - academic.oup.com
Major life transitions are always difficult because change costs energy. Recent findings have
demonstrated how mitochondrial oxidative phosphorylation (OxPhos) defects increase the …