[HTML][HTML] MitophAging: mitophagy in aging and disease

D Bakula, M Scheibye-Knudsen - Frontiers in cell and developmental …, 2020 - frontiersin.org
Maintaining mitochondrial health is emerging as a keystone in aging and associated
diseases. The selective degradation of mitochondria by mitophagy is of particular …

Current treatment options and novel nanotechnology-driven enzyme replacement strategies for lysosomal storage disorders

A Del Grosso, G Parlanti, R Mezzena… - Advanced Drug Delivery …, 2022 - Elsevier
Lysosomal storage disorders (LSDs) are a vast group of more than 50 clinically identified
metabolic diseases. They are singly rare, but they affect collectively 1 on 5,000 live births …

[HTML][HTML] Lipophagy and lipolysis status in lipid storage and lipid metabolism diseases

A Kloska, M Węsierska, M Malinowska… - International journal of …, 2020 - mdpi.com
This review discusses how lipophagy and cytosolic lipolysis degrade cellular lipids, as well
as how these pathway ys communicate, how they affect lipid metabolism and energy …

Brain-targeted enzyme-loaded nanoparticles: A breach through the blood-brain barrier for enzyme replacement therapy in Krabbe disease

A Del Grosso, M Galliani, L Angella, M Santi… - Science …, 2019 - science.org
Lysosomal storage disorders (LSDs) result from an enzyme deficiency within lysosomes.
The systemic administration of the missing enzyme, however, is not effective in the case of …

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] FDA-approved kinase inhibitors in preclinical and clinical trials for neurological disorders

A Lui, J Vanleuven, D Perekopskiy, D Liu, D Xu… - Pharmaceuticals, 2022 - mdpi.com
Cancers and neurological disorders are two major types of diseases. We previously
developed a new concept termed “Aberrant Cell Cycle Diseases”(ACCD), revealing that …

[HTML][HTML] Activities of (poly) phenolic antioxidants and other natural autophagy modulators in the treatment of Sanfilippo disease: remarkable efficacy of resveratrol in …

E Rintz, M Podlacha, Z Cyske, K Pierzynowska… - …, 2023 - Elsevier
Sanfilippo disease, caused by mutations in the genes encoding heparan sulfate (HS)(a
glycosaminoglycan; GAG) degradation enzymes, is a mucopolysaccharidosis (MPS), which …

[HTML][HTML] Chronic Rapamycin administration via drinking water mitigates the pathological phenotype in a Krabbe disease mouse model through autophagy activation

A Del Grosso, S Carpi, M De Sarlo, L Scaccini… - Biomedicine & …, 2024 - Elsevier
Krabbe disease (KD) is a rare disorder arising from the deficiency of the lysosomal enzyme
galactosylceramidase (GALC), leading to the accumulation of the cytotoxic metabolite …

[HTML][HTML] Rapamycin alleviates protein aggregates, reduces neuroinflammation, and rescues demyelination in globoid Cell leukodystrophy

DS Lin, YW Huang, TH Lee, L Chang, ZD Huang… - Cells, 2023 - mdpi.com
We have shown in vivo and in vitro previously that psychosine causes dysfunction of
autophagy and the ubiquitin-proteasome system underlying the pathogenesis of globoid cell …

[HTML][HTML] Impaired autophagy in krabbe disease: the role of BCL2 and beclin-1 phosphorylation

N Papini, R Todisco, P Giussani, M Dei Cas… - International Journal of …, 2023 - mdpi.com
Autophagic impairment was identified in many lysosomal storage diseases and adult
neurodegenerative diseases. It seems that this defect could be directly related to the …