Disease‐specific phenotypes in dopamine neurons from human iPS‐based models of genetic and sporadic Parkinson's disease

Sánchez‐Danés, Adriana; Richaud‐Patín, Yvonne; Carballo‐Carbajal, Iria; Jiménez‐Delgado, Senda; Caig, Carles; Mora, Sergio; Guglielmo, Claudia Di; Ezquerra, Mario; Patel, Bindiben; Giralt, Albert; Canals, Josep M.; Memo, Maurizio; Alberch, Jordi; López‐Barneo, José; Vila, Miquel; Cuervo, Ana María; Tolosa, Eduard; Consiglio, Antonella; Raya, Ángel

doi:10.1002/emmm.201200215

articleEMBO Molecular MedicineMar 8, 2012GOLD OA

Disease‐specific phenotypes in dopamine neurons from human iPS‐based models of genetic and sporadic Parkinson's disease

ASAdriana Sánchez‐Danés YRYvonne Richaud‐Patín ICIria Carballo‐Carbajal SJSenda Jiménez‐Delgado CCCarles Caig

Institute for Research in Biomedicine · Universitat de Barcelona · +12 more institutions

PubMed

Indexed incrossrefdoajpubmed

Abstract

Induced pluripotent stem cells (iPSC) offer an unprecedented opportunity to model human disease in relevant cell types, but it is unclear whether they could successfully model age-related diseases such as Parkinson's disease (PD). Here, we generated iPSC lines from seven patients with idiopathic PD (ID-PD), four patients with familial PD associated to the G2019S mutation in the Leucine-Rich Repeat Kinase 2 (LRRK2) gene (LRRK2-PD) and four age- and sex-matched healthy individuals (Ctrl). Over long-time culture, dopaminergic neurons (DAn) differentiated from either ID-PD- or LRRK2-PD-iPSC showed morphological alterations, including reduced numbers of neurites and neurite arborization, as well as accumulation of…

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Topics

Keywords

LRRK2
Induced pluripotent stem cell
Neurite
Autophagy
Parkinson's disease
Biology
Pathogenesis
Disease

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