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Article

Friedreich ataxia-induced pluripotent stem cell-derived neurons show a cellular phenotype that is corrected by a benzamide HDAC inhibitor.

Human molecular genetics - 15 Nov 2016

Codazzi Franca, Hu Amelié, Rai Myriam, Donatello Simona, Salerno Scarzella Floramarida, Mangiameli Elisabeth, Pelizzoni Ilaria, Grohovaz Fabio, Pandolfo Massimo

Abstract excerpt

We employed induced pluripotent stem cell (iPSC)-derived neurons obtained from Friedreich ataxia (FRDA) patients and healthy subjects, FRDA neurons and CT neurons, respectively, to unveil phenotypic alterations related to frataxin (FXN) deficiency and investigate if they can be reversed by treatments that upregulate FXN. FRDA and control iPSCs were equally capable of differentiating into a neuronal or astrocytic...

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Source

Identifiers

PMID: 28175303
DOI: 10.1093/hmg/ddw308

Genes

FXN
SOD2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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