Article

<i>C9orf72</i> -derived poly-GA DPRs undergo endocytic uptake in iNPC-derived astrocytes and spread to motor neurons

2021-10-12

Abstract excerpt

Dipeptide repeat proteins (DPRs) are aggregation-prone polypeptides encoded by the pathogenic G4C2 repeat expansion in the C9orf72 gene, the most common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD). In this study, we focus on the role of poly-GA DPRs in disease spread. We demonstrate that recombinant poly-GA oligomers can directly convert into solid-like aggregates and form...

Topics

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

Identifiers and source

Literature Corpus work: aee31dbe-3ebd-5ab7-a258-840304f865b0
DOI: 10.1101/2021.10.11.463891

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

<i>C9orf72</i> -derived poly-GA DPRs undergo endocytic uptake in iNPC-derived astrocytes and spread to motor neuronsDOI 10.1101/2021.10.11.463891Read

Cell‐to‐cell transmission of C9orf72 poly‐(Gly‐Ala) triggers key features of ALS/FTDSemantic proximity Read

Cell-to-Cell Transmission of Dipeptide Repeat Proteins Linked to C9orf72-ALS/FTDSemantic proximity Read

The molecular mechanism of uptake and cell-to-cell transmission of arginine-containing dipeptide repeat proteinsSemantic proximity Read

Glial control of extracellular α-synuclein propagation in LRRK2 Parkinson's disease.Semantic proximity Read

Poly-GP accumulation due to C9orf72 loss of function induces motor neuron apoptosis through autophagy and mitophagy defectsSemantic proximity Read

GBA Mutations Influence the Release and Pathological Effects of Small Extracellular Vesicles from Fibroblasts of Patients with Parkinson's Disease.Semantic proximity Read

C9orf72 dipeptides disrupt the nucleocytoplasmic transport machinery and cause TDP-43 mislocalisation to the cytoplasmSemantic proximity Read

Comprehensive evaluation of human-derived anti-poly-GA antibodies in cellular and animal models of C9orf72 diseaseSemantic proximity Read

Glucosylceramide-induced ectosomes propagate pathogenic α-synuclein in Parkinson's disease.Semantic proximity Read

Parkinson’s Disease-Associated LRRK2 Interferes with Astrocyte-Mediated Alpha-Synuclein ClearanceSemantic proximity Read

Glucosylceramide induced ectosomes propagate pathogenic α-synuclein in Parkinson’s diseaseSemantic proximity Read

Comprehensive preclinical evaluation of human-derived anti-poly-GA antibodies in cellular and animal models of C9ORF72 diseaseSemantic proximity Read