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PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in <i>C9orf72</i> ALS/FTD neurons

2023-07-17

Abstract excerpt

A GGGGCC repeat expansion in C9orf72 is the most common genetic cause of ALS and FTD (C9ALS/FTD). The presence of dipeptide repeat (DPR) proteins, generated by translation of the expanded repeat, is a major pathogenic feature of C9ALS/FTD pathology, but their most relevant effects in a physiological context are not known. Here, we generated C9orf72 DPR knock-in mouse models characterised by physiological express...

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Literature Corpus work
88d70459-577c-58d0-87e3-8f9e6d964d8d
DOI
10.1101/2023.07.17.549331
Open publication

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PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in <i>C9orf72</i> ALS/FTD neuronsDOI 10.1101/2023.07.17.549331
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