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Neural Stem Cells of Parkinson's Disease Patients Exhibit Aberrant Mitochondrial Morphology and Functionality.

Abstract：

:Emerging evidence suggests that Parkinson's disease (PD), besides being an age-associated disorder, might also have a neurodevelopment component. Disruption of mitochondrial homeostasis has been highlighted as a crucial cofactor in its etiology. Here, we show that PD patient-specific human neuroepithelial stem cells (NESCs), carrying the LRRK2-G2019S mutation, recapitulate key mitochondrial defects previously described only in differentiated dopaminergic neurons. By combining high-content imaging approaches, 3D image analysis, and functional mitochondrial readouts we show that LRRK2-G2019S mutation causes aberrations in mitochondrial morphology and functionality compared with isogenic controls. LRRK2-G2019S NESCs display an increased number of mitochondria compared with isogenic control lines. However, these mitochondria are more fragmented and exhibit decreased membrane potential. Functional alterations in LRRK2-G2019S cultures are also accompanied by a reduced mitophagic clearance via lysosomes. These findings support the hypothesis that preceding mitochondrial developmental defects contribute to the manifestation of the PD pathology later in life.

journal_name

Stem Cell Reports

journal_title

Stem cell reports

authors

Walter J,Bolognin S,Antony PMA,Nickels SL,Poovathingal SK,Salamanca L,Magni S,Perfeito R,Hoel F,Qing X,Jarazo J,Arias-Fuenzalida J,Ignac T,Monzel AS,Gonzalez-Cano L,Pereira de Almeida L,Skupin A,Tronstad KJ,Schwamborn

doi

10.1016/j.stemcr.2019.03.004

subject

Has Abstract

pub_date

2019-05-14 00:00:00

pages

878-889

issue

5

issn

2213-6711

pii

S2213-6711(19)30089-X

journal_volume

12

pub_type

杂志文章

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