Emerging trends in the field of inflammation and proteinopathy in ALS/FTD spectrum disorder

F De Marchi, T Franjkic, P Schito, T Russo, J Nimac… - Biomedicines, 2023 - mdpi.com
Proteinopathy and neuroinflammation are two main hallmarks of neurodegenerative
diseases. They also represent rare common events in an exceptionally broad landscape of …

Defects of nutrient signaling and autophagy in neurodegeneration

J Ondaro, H Hernandez-Eguiazu… - Frontiers in Cell and …, 2022 - frontiersin.org
Neurons are post-mitotic cells that allocate huge amounts of energy to the synthesis of new
organelles and molecules, neurotransmission and to the maintenance of redox …

ERO1A inhibition mitigates neuronal ER stress and ameliorates UBQLN2ALS phenotypes in Drosophila melanogaster

R Yeewa, A Sangphukieo, P Jantaree… - Progress in …, 2024 - Elsevier
Modulating the ER stress pathway holds therapeutic promise for neurodegenerative
diseases; however, identifying optimal targets remains challenging. In this study, we …

Artificial enforcement of the unfolded protein response (UPR) reduces disease features in multiple preclinical models of ALS/FTD.

V Valenzuela, D Becerra, JI Astorga, M Fuentealba… - Molecular Therapy, 2025 - cell.com
Amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (FTD) are part of a
spectrum of diseases that share several causative genes, resulting in a combinatory of motor …

Chronological and Biological Aging in Amyotrophic Lateral Sclerosis and the Potential of Senolytic Therapies

AR Dashtmian, FB Darvishi, WD Arnold - Cells, 2024 - mdpi.com
Amyotrophic Lateral Sclerosis (ALS) is a group of sporadic and genetic neurodegenerative
disorders that result in losses of upper and lower motor neurons. Treatment of ALS is limited …

The therapeutic potential of dibenzoylmethane (DBM) in a C9ORF72-mediated FTD/ALS mouse model

PM Torres Corales - 2022 - repositorio.uchile.cl
The most frequent genetic cause of C9FTD/ALS is a hexanucleotide repeat expansion in the
C9ORF72 gene. This mutation produces dipeptide repeats (DPR) that are neurotoxic and …