Mitochondria in health, disease, and aging

JS Harrington, SW Ryter, M Plataki… - Physiological …, 2023 - journals.physiology.org
Mitochondria are well known as organelles responsible for the maintenance of cellular
bioenergetics through the production of ATP. Although oxidative phosphorylation may be …

Amyotrophic lateral sclerosis: translating genetic discoveries into therapies

F Akçimen, ER Lopez, JE Landers, A Nath… - Nature Reviews …, 2023 - nature.com
Recent advances in sequencing technologies and collaborative efforts have led to
substantial progress in identifying the genetic causes of amyotrophic lateral sclerosis (ALS) …

PINK1/Parkin-mediated mitophagy in neurodegenerative diseases

J Li, D Yang, Z Li, M Zhao, D Wang, Z Sun, P Wen… - Ageing research …, 2023 - Elsevier
Mitochondria play key roles in bioenergetics, metabolism, and signaling; therefore, stable
mitochondrial function is essential for cell survival, particularly in energy-intensive neuronal …

Antisense oligonucleotide silencing of FUS expression as a therapeutic approach in amyotrophic lateral sclerosis

VA Korobeynikov, AK Lyashchenko… - Nature medicine, 2022 - nature.com
Fused in sarcoma (FUS) is an RNA-binding protein that is genetically and pathologically
associated with rare and aggressive forms of amyotrophic lateral sclerosis (ALS) and …

Amyotrophic lateral sclerosis: a clinical review

P Masrori, P Van Damme - European journal of neurology, 2020 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting primarily the
motor system, but in which extra‐motor manifestations are increasingly recognized. The loss …

ALS genetics, mechanisms, and therapeutics: where are we now?

R Mejzini, LL Flynn, IL Pitout, S Fletcher… - Frontiers in …, 2019 - frontiersin.org
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …

[HTML][HTML] Phase separation and neurodegenerative diseases: a disturbance in the force

A Zbinden, M Pérez-Berlanga, P De Rossi… - Developmental cell, 2020 - cell.com
Protein aggregation is the main hallmark of neurodegenerative diseases. Many proteins
found in pathological inclusions are known to undergo liquid-liquid phase separation, a …

Molecular and cellular mechanisms of selective vulnerability in neurodegenerative diseases

M Kampmann - Nature Reviews Neuroscience, 2024 - nature.com
The selective vulnerability of specific neuronal subtypes is a hallmark of neurodegenerative
diseases. In this Review, I summarize our current understanding of the brain regions and …

Stress granules and neurodegeneration

B Wolozin, P Ivanov - Nature Reviews Neuroscience, 2019 - nature.com
Recent advances suggest that the response of RNA metabolism to stress has an important
role in the pathophysiology of neurodegenerative diseases, particularly amyotrophic lateral …

Beyond aggregation: Pathological phase transitions in neurodegenerative disease

C Mathieu, RV Pappu, JP Taylor - Science, 2020 - science.org
Over the past decade, phase transitions have emerged as a fundamental mechanism of
cellular organization. In parallel, a wealth of evidence has accrued indicating that …