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Human pluripotent-stem-cell-derived organoids for drug discovery and evaluation
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 …
new era for disease modeling and drug discovery. Over the past decade, significant …
[HTML][HTML] Regulatory roles of mitochondria and metabolism in neurogenesis
Neural stem cells (NSCs) undergo massive molecular and cellular changes during neuronal
differentiation. These include mitochondria and metabolism remodelling, which were …
differentiation. These include mitochondria and metabolism remodelling, which were …
OxPhos defects cause hypermetabolism and reduce lifespan in cells and in patients with mitochondrial diseases
Patients with primary mitochondrial oxidative phosphorylation (OxPhos) defects present with
fatigue and multi-system disorders, are often lean, and die prematurely, but the mechanistic …
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
Highlights•The human cerebral cortex is characterized by a protracted post-natal
development (neoteny) compared with other species.•The species-specific rate of cortical …
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 …
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
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 …
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 …
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 …
mimic the structure and physiology of human organs. Patient-specific induced pluripotent …
Mitochondria in neurogenesis: Implications for mitochondrial diseases
Mitochondria are organelles with recognized key roles in cellular homeostasis, including
bioenergetics, redox, calcium signaling, and cell death. Mitochondria are essential for …
bioenergetics, redox, calcium signaling, and cell death. Mitochondria are essential for …
The energetics of cellular life transitions
Major life transitions are always difficult because change costs energy. Recent findings have
demonstrated how mitochondrial oxidative phosphorylation (OxPhos) defects increase the …
demonstrated how mitochondrial oxidative phosphorylation (OxPhos) defects increase the …