Molecular mechanisms of cell death in neurological diseases

D Moujalled, A Strasser, JR Liddell - Cell Death & Differentiation, 2021 - nature.com
Tightly orchestrated programmed cell death (PCD) signalling events occur during normal
neuronal development in a spatially and temporally restricted manner to establish the neural …

The unfolded protein response: controlling cell fate decisions under ER stress and beyond

C Hetz - Nature reviews Molecular cell biology, 2012 - nature.com
Protein-folding stress at the endoplasmic reticulum (ER) is a salient feature of specialized
secretory cells and is also involved in the pathogenesis of many human diseases. ER stress …

Neuronal cell death

M Fricker, AM Tolkovsky, V Borutaite… - Physiological …, 2018 - journals.physiology.org
Neuronal cell death occurs extensively during development and pathology, where it is
especially important because of the limited capacity of adult neurons to proliferate or be …

[HTML][HTML] Autophagy and apoptosis dysfunction in neurodegenerative disorders

S Ghavami, S Shojaei, B Yeganeh, SR Ande… - Progress in …, 2014 - Elsevier
Autophagy and apoptosis are basic physiologic processes contributing to the maintenance
of cellular homeostasis. Autophagy encompasses pathways that target long-lived cytosolic …

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

C Hetz, B Mollereau - Nature Reviews Neuroscience, 2014 - nature.com
The unfolded protein response (UPR) is a homeostatic mechanism by which cells regulate
levels of misfolded proteins in the endoplasmic reticulum (ER). Although it is well …

Gasdermin-E mediates mitochondrial damage in axons and neurodegeneration

DV Neel, H Basu, G Gunner, MD Bergstresser… - Neuron, 2023 - cell.com
Mitochondrial dysfunction and axon loss are hallmarks of neurologic diseases. Gasdermin
(GSDM) proteins are executioner pore-forming molecules that mediate cell death, yet their …

PUMA, a potent killer with or without p53

J Yu, L Zhang - Oncogene, 2008 - nature.com
PUMA (p53 upregulated modulator of apoptosis) is a Bcl-2 homology 3 (BH3)-only Bcl-2
family member and a critical mediator of p53-dependent and-independent apoptosis …

XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

C Hetz, P Thielen, S Matus, M Nassif… - Genes & …, 2009 - genesdev.cshlp.org
Mutations in superoxide dismutase-1 (SOD1) cause familial amyotrophic lateral sclerosis
(fALS). Recent evidence implicates adaptive responses to endoplasmic reticulum (ER) …

Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS

BJ Turner, K Talbot - Progress in neurobiology, 2008 - Elsevier
Gain-of-function mutations in the Cu, Zn-superoxide dismutase (SOD1) gene are implicated
in progressive motor neuron death and paralysis in one form of inherited amyotrophic lateral …

Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons

K Castillo, M Nassif, V Valenzuela, F Rojas, S Matus… - Autophagy, 2013 - Taylor & Francis
Amyotrophic lateral sclerosis (ALS) is a fatal motoneuron disease with no current effective
treatment. Accumulation of abnormal protein inclusions containing SOD1, TARDBP, FUS …