Autophagy gone awry in neurodegenerative diseases

E Wong, AM Cuervo - Nature neuroscience, 2010 - nature.com
Autophagy is essential for neuronal homeostasis, and its dysfunction has been directly
linked to a growing number of neurodegenerative disorders. The reasons behind …

Polyglutamine (PolyQ) Diseases: Navigating the Landscape of Neurodegeneration

R Tenchov, JM Sasso, QA Zhou - ACS Chemical Neuroscience, 2024 - ACS Publications
Polyglutamine (polyQ) diseases are a group of inherited neurodegenerative disorders
caused by expanded cytosine-adenine-guanine (CAG) repeats encoding proteins with …

Aggrephagy: selective disposal of protein aggregates by macroautophagy

T Lamark, T Johansen - International journal of cell biology, 2012 - Wiley Online Library
Protein aggregation is a continuous process in our cells. Some proteins aggregate in a
regulated manner required for different vital functional processes in the cells whereas other …

Repeat expansion disease: progress and puzzles in disease pathogenesis

AR La Spada, JP Taylor - Nature Reviews Genetics, 2010 - nature.com
Repeat expansion mutations cause at least 22 inherited neurological diseases. The
complexity of repeat disease genetics and pathobiology has revealed unexpected shared …

Autophagy and the immune system

P Kuballa, WM Nolte, AB Castoreno… - Annual review of …, 2012 - annualreviews.org
Stressors ranging from nutrient deprivation to immune signaling can induce the degradation
of cytoplasmic material by a process known as autophagy. Increasingly, research on …

Post-translational modifications: regulators of neurodegenerative proteinopathies

R Gupta, M Sahu, D Srivastava, S Tiwari… - Ageing Research …, 2021 - Elsevier
One of the hallmark features in the neurodegenerative disorders (NDDs) is the accumulation
of aggregated and/or non-functional protein in the cellular milieu. Post-translational …

Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias

A Matilla-Dueñas, T Ashizawa, A Brice, S Magri… - The Cerebellum, 2014 - Springer
Intensive scientific research devoted in the recent years to understand the molecular
mechanisms or neurodegeneration in spinocerebellar ataxias (SCAs) are identifying new …

[HTML][HTML] Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease

JP Miller, J Holcomb, I Al-Ramahi, M De Haro, J Gafni… - Neuron, 2010 - cell.com
Proteolytic cleavage of huntingtin (Htt) is known to be a key event in the pathogenesis of
Huntington's disease (HD). Our understanding of proteolytic processing of Htt has thus far …

Proteolysis of Ambra1 during apoptosis has a role in the inhibition of the autophagic pro-survival response

V Pagliarini, E Wirawan, A Romagnoli… - Cell Death & …, 2012 - nature.com
Under stress conditions, pro-survival and pro-death processes are concomitantly activated
and the final outcome depends on the complex crosstalk between these pathways. In most …

Autophagy in the central nervous system: implications for neurodegenerative disorders

M **louri, L Stefanis - … (Formerly Current Drug Targets-CNS & …, 2010 - ingentaconnect.com
The autophagy-lysosomal pathway is a major proteolytic pathway that in mammalian
systems mainly comprises of macroautophagy and chaperone-mediated autophagy. The …