Article

Mutant SOD1 expressed by oligodendrocytes aggregates in myelinic nanochannels and accelerates disease progression in familial ALS mice

2026-06-11

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a highly debilitating and fatal disease characterized by the progressive loss of motor neurons. Reduced oligodendroglial support has been implicated in ALS progression but remains mechanistically unexplained. Here, using a mutant superoxide dismutase 1 (SOD1-G37R) mouse model of familial ALS, Cre-mediated excision of the mutant SOD1 gene within the oligodendrocyte lineage pri...

Topics

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

Identifiers and source

Literature Corpus work: 21c7c5bc-f755-5fbb-8e25-fa983a6c6b23
DOI: 10.64898/2026.06.09.731100

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Mutant SOD1 expressed by oligodendrocytes aggregates in myelinic nanochannels and accelerates disease progression in familial ALS miceDOI 10.64898/2026.06.09.731100Read

Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities.Semantic proximity Read

Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice.Semantic proximity Read

Alteration of Mitochondrial Integrity as Upstream Event in the Pathophysiology of SOD1-ALSSemantic proximity Read

The mitochondrial permeability transition pore in motor neurons: involvement in the pathobiology of ALS mice.Semantic proximity Read

Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis.Semantic proximity Read

Beyond Neurodegeneration: White Matter Vacuolation as a Primary Myelin DefectSemantic proximity Read

In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space.Semantic proximity Read

Skeletal myotubes expressing ALS mutant SOD1 induce pathogenic changes, impair mitochondrial axonal transport, and trigger motoneuron death.Semantic proximity Read

Aggregates of mutant protein appear progressively in dendrites, in periaxonal processes of oligodendrocytes, and in neuronal and astrocytic perikarya of mice expressing the SOD1(G93A) mutation of familial amyotrophic lateral sclerosis.Semantic proximity Read

Oligomerization of mutant SOD1 in mitochondria of motoneuronal cells drives mitochondrial damage and cell toxicity.Semantic proximity Read

Impaired nucleocytoplasmic transport in SOD1-mediated ALS.Semantic proximity Read

Human Motor Neurons With SOD1-G93A Mutation Generated From CRISPR/Cas9 Gene-Edited iPSCs Develop Pathological Features of Amyotrophic Lateral SclerosisSemantic proximity Read