Promoting the clearance of neurotoxic proteins in neurodegenerative disorders of ageing
Neurodegenerative disorders of ageing (NDAs) such as Alzheimer disease, Parkinson
disease, frontotemporal dementia, Huntington disease and amyotrophic lateral sclerosis …
disease, frontotemporal dementia, Huntington disease and amyotrophic lateral sclerosis …
Stress granules, RNA-binding proteins and polyglutamine diseases: too much aggregation?
A Marcelo, R Koppenol, LP de Almeida, CA Matos… - Cell death & …, 2021 - nature.com
Stress granules (SGs) are membraneless cell compartments formed in response to different
stress stimuli, wherein translation factors, mRNAs, RNA-binding proteins (RBPs) and other …
stress stimuli, wherein translation factors, mRNAs, RNA-binding proteins (RBPs) and other …
Autophagy induction as a therapeutic strategy for neurodegenerative diseases
Autophagy is a major, conserved cellular pathway by which cells deliver cytoplasmic
contents to lysosomes for degradation. Genetic studies have revealed extensive links …
contents to lysosomes for degradation. Genetic studies have revealed extensive links …
PINK1 and BECN1 relocalize at mitochondria-associated membranes during mitophagy and promote ER-mitochondria tethering and autophagosome formation
V Gelmetti, P De Rosa, L Torosantucci, ES Marini… - Autophagy, 2017 - Taylor & Francis
Mitophagy is a highly specialized process to remove dysfunctional or superfluous
mitochondria through the macroautophagy/autophagy pathway, aimed at protecting cells …
mitochondria through the macroautophagy/autophagy pathway, aimed at protecting cells …
Mutation in ATG5 reduces autophagy and leads to ataxia with developmental delay
Autophagy is required for the homeostasis of cellular material and is proposed to be
involved in many aspects of health. Defects in the autophagy pathway have been observed …
involved in many aspects of health. Defects in the autophagy pathway have been observed …
Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice
HS McLoughlin, LR Moore, R Chopra… - Annals of …, 2018 - Wiley Online Library
Objective Spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease,
is the most common dominantly inherited ataxia. Despite advances in understanding this …
is the most common dominantly inherited ataxia. Despite advances in understanding this …
Machado–Joseph disease/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapy
CA Matos, LP de Almeida… - Journal of …, 2019 - Wiley Online Library
Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA 3), is an incurable disorder, widely regarded as the most common form of …
(SCA 3), is an incurable disorder, widely regarded as the most common form of …
Autophagic and endo-lysosomal dysfunction in neurodegenerative disease
Due to their post-mitotic state, metabolic demands and often large polarised morphology, the
function and survival of neurons is dependent on an efficient cellular waste clearance …
function and survival of neurons is dependent on an efficient cellular waste clearance …
Clinical gene therapy for neurodegenerative diseases: past, present, and future
Clinical gene therapy has made important advances over the last decade. Among
neurological diseases, severe genetic neurodegenerative conditions have been the focus of …
neurological diseases, severe genetic neurodegenerative conditions have been the focus of …
Targeting autophagy in neurodegenerative diseases
The most prevalent neurodegenerative disorders involve protein misfolding and the
aggregation of specific proteins. Autophagy is becoming an attractive target to treat …
aggregation of specific proteins. Autophagy is becoming an attractive target to treat …