Mitochondrial function and dynamics in neural stem cells and neurogenesis: Implications for neurodegenerative diseases

P Coelho, L Fão, S Mota, AC Rego - Ageing research reviews, 2022 - Elsevier
Mitochondria have been largely described as the powerhouse of the cell and recent findings
demonstrate that this organelle is fundamental for neurogenesis. The mechanisms …

Polymer-based drug delivery systems under investigation for enzyme replacement and other therapies of lysosomal storage disorders

M Placci, MI Giannotti, S Muro - Advanced drug delivery reviews, 2023 - Elsevier
Lysosomes play a central role in cellular homeostasis and alterations in this compartment
associate with many diseases. The most studied example is that of lysosomal storage …

Mechanisms of demyelination and neurodegeneration in globoid cell leukodystrophy

ML Feltri, NI Weinstock, J Favret, N Dhimal, L Wrabetz… - Glia, 2021 - Wiley Online Library
Globoid cell leukodystrophy (GLD), also known as Krabbe disease, is a lysosomal storage
disorder causing extensive demyelination in the central and peripheral nervous systems …

[HTML][HTML] Models to study basic and applied aspects of lysosomal storage disorders

Á Gaudioso, TP Silva, MD Ledesma - Advanced Drug Delivery Reviews, 2022 - Elsevier
The lack of available treatments and fatal outcome in most lysosomal storage disorders
(LSDs) have spurred research on pathological mechanisms and novel therapies in recent …

Fabry disease and central nervous system involvement: from big to small, from brain to synapse

E Cortés-Saladelafont, J Fernández-Martín… - International Journal of …, 2023 - mdpi.com
Fabry disease (FD) is an X-linked lysosomal storage disorder (LSD) secondary to mutations
in the GLA gene that causes dysfunctional activity of lysosomal hydrolase α-galactosidase A …

Free sialic acid storage disorder: Progress and promise

M Huizing, ME Hackbarth, DR Adams, M Wasserstein… - Neuroscience …, 2021 - Elsevier
Lysosomal free sialic acid storage disorder (FSASD) is an extremely rare, autosomal
recessive, neurodegenerative, multisystemic disorder caused by defects in the lysosomal …

Human iPSC-derived neural stem cells display a radial glia-like signature in vitro and favorable long-term safety in transplanted mice

M Luciani, C Garsia, S Beretta, L Petiti, C Peano… - bioRxiv, 2023 - biorxiv.org
Human induced pluripotent stem cell-derived neural stem/progenitor cells (hiPSC-NSCs)
are a promising source for cell therapy approaches to treat neurodegenerative and …

Delineating the neuropathology of lysosomal storage diseases using patient-derived induced pluripotent stem cells

KR Sabitha, D Chandran, AK Shetty… - Stem Cells and …, 2022 - liebertpub.com
Lysosomal storage diseases (LSDs) are inherited metabolic diseases caused by deficiency
of lysosomal enzymes, essential for the normal development of the brain and other organs …

Promising developments in the use of induced pluripotent stem cells in research of ADHD

CM Yde Ohki, RV McNeill, M Nieberler… - New Discoveries in the …, 2022 - Springer
Although research using animal models, peripheral and clinical biomarkers, multimodal
neuroimaging techniques and (epi) genetic information has advanced our understanding of …

Intracerebroventricular transplantation of human iPSC-derived neural stem cells (hiPSC-NSCs) into neonatal mice

M Luciani, C Garsia, E Mangiameli, V Meneghini… - Methods in Cell …, 2022 - Elsevier
Human neural stem cells (hNSCs) hold great promises for the development of cell-based
therapies for neurodegenerative diseases, given their capability to provide …