Article

GCN2 inhibition reduces mutant SOD1 clustering and toxicity and delays disease progression in an Amyotrophic Lateral Sclerosis mouse model

2024-07-01

Abstract excerpt

<title>Abstract</title> <p>The disruption of protein folding homeostasis in motoneurons (MNs), and the ensuing accumulation of protein aggregates, is one of the main molecular hallmarks of amyotrophic lateral sclerosis (ALS) pathology, and has been recapitulated in cellular and animal disease models. The loss of proteostasis and other stresses in the MN trigger the activation of a general stress mechanism, the in...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 8eb401c5-1c61-5de0-8c1e-ba40dce6eb5f
DOI: 10.21203/rs.3.rs-4544133/v1

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

GCN2 inhibition reduces mutant SOD1 clustering and toxicity and delays disease progression in an Amyotrophic Lateral Sclerosis mouse modelDOI 10.21203/rs.3.rs-4544133/v1Read

<i>Atxn2</i> -CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppressionSemantic proximity Read

An enhanced integrated stress response ameliorates mutant SOD1-induced ALSSemantic proximity Read

Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS.Semantic proximity Read

Analyzing ER stress response in ALS patient derived motor neurons identifies druggable neuroprotective targetsSemantic proximity Read

Integrative proteomics highlight presynaptic alterations and c-Jun misactivation as convergent pathomechanisms in ALSSemantic proximity Read

PAD2 knockout reduces myelin protein aggregates, modulates neuroinflammation and protects motor neurons, axons and neuromuscular junction in a SOD1-ALS mouse modelSemantic proximity Read

TECPR2 maintains mitochondrial homeostasis in neurodegenerationSemantic proximity Read

Stem cell-derived cranial and spinal motor neurons reveal proteostatic differences between ALS resistant and sensitive motor neuronsSemantic proximity Read

Fine tuning of the unfolded protein response by ISRIB improves neuronal survival in a model of amyotrophic lateral sclerosis.Semantic proximity Read

Unveiling the double-edged sword: SOD1 trimers possess tissue-selective toxicity and bind septin-7 in motor neuron-like cellsSemantic proximity Read

Early interneuron dysfunction in ALS: Insights from a mutant sod1 zebrafish modelSemantic proximity Read

Selective removal of misfolded SOD1 delays disease onset in a mouse model of amyotrophic lateral sclerosisSemantic proximity Read