Chunk #23 — Results — Oligodendrocytes Derived from Mutant C9ORF72 Patients Express Intranuclear RNA Foci, but do not Exhibit Impairment in Maturation

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Maturation and electrophysiological properties of human pluripotent stem cell-derived oligodendrocytes.

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Having established benchmark physiological and maturation features of control OPCs and to assess the maturation profile of oligodendrocytes in a disease model we next derived oligodendroglia from hPSCs obtained from two ALS patients (iPSC91 and iPSC92) harboring mutations in the C9ORF72 gene (Fig. 7A). Mutations in the C9ORF72 gene are the most common manifestation of familial ALS and contribute to 10% of sporadic cases 37, 38.