Parkin and PINK1 Patient iPSC-Derived Midbrain Dopamine Neurons Exhibit Mitochondrial Dysfunction and alpha-Synuclein Accumulation
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Chung, Sun Young | - |
dc.contributor.author | Kishinevsky, Sarah | - |
dc.contributor.author | Mazzulli, Joseph R. | - |
dc.contributor.author | Graziotto, John | - |
dc.contributor.author | Mrejeru, Ana | - |
dc.contributor.author | Mosharov, Eugene V. | - |
dc.contributor.author | Puspita, Lesly | - |
dc.contributor.author | Valiulahi, Parvin | - |
dc.contributor.author | Sulzer, David | - |
dc.contributor.author | Milner, Teresa A. | - |
dc.contributor.author | Taldone, Tony | - |
dc.contributor.author | Krainc, Dimitri | - |
dc.contributor.author | Studer, Lorenz | - |
dc.contributor.author | Shim, Jae-Won | - |
dc.date.accessioned | 2021-08-11T16:45:23Z | - |
dc.date.available | 2021-08-11T16:45:23Z | - |
dc.date.issued | 2016-10-11 | - |
dc.identifier.issn | 2213-6711 | - |
dc.identifier.uri | https://scholarworks.bwise.kr/sch/handle/2021.sw.sch/8680 | - |
dc.description.abstract | Parkinson's disease (PD) is characterized by the selective loss of dopamine neurons in the substantia nigra; however, the mechanism of neurodegeneration in PD remains unclear. A subset of familial PD is linked to mutations in PARK2 and PINK1, which lead to dysfunctional mitochondria-related proteins Parkin and PINK1, suggesting that pathways implicated in these monogenic forms could play a more general role in PD. We demonstrate that the identification of disease-related phenotypes in PD-patient-specific induced pluripotent stem cell (iPSC)-derived midbrain dopamine (mDA) neurons depends on the type of differentiation protocol utilized. In a floor-plate-based but not a neural-rosette-based directed differentiation strategy, iPSC-derived mDA neurons recapitulate PD phenotypes, including pathogenic protein accumulation, cell-type-specific vulnerability, mitochondrial dysfunction, and abnormal neurotransmitter homeostasis. We propose that these form a pathogenic loop that contributes to disease. Our study illustrates the promise of iPSC technology for examining PD pathogenesis and identifying therapeutic targets. | - |
dc.format.extent | 14 | - |
dc.language | 영어 | - |
dc.language.iso | ENG | - |
dc.publisher | Cell Press | - |
dc.title | Parkin and PINK1 Patient iPSC-Derived Midbrain Dopamine Neurons Exhibit Mitochondrial Dysfunction and alpha-Synuclein Accumulation | - |
dc.type | Article | - |
dc.publisher.location | 미국 | - |
dc.identifier.doi | 10.1016/j.stemcr.2016.08.012 | - |
dc.identifier.scopusid | 2-s2.0-84992183855 | - |
dc.identifier.wosid | 000389508600007 | - |
dc.identifier.bibliographicCitation | Stem Cell Reports, v.7, no.4, pp 664 - 677 | - |
dc.citation.title | Stem Cell Reports | - |
dc.citation.volume | 7 | - |
dc.citation.number | 4 | - |
dc.citation.startPage | 664 | - |
dc.citation.endPage | 677 | - |
dc.type.docType | Article | - |
dc.description.isOpenAccess | N | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
dc.relation.journalResearchArea | Cell Biology | - |
dc.relation.journalWebOfScienceCategory | Cell & Tissue Engineering | - |
dc.relation.journalWebOfScienceCategory | Cell Biology | - |
dc.subject.keywordPlus | PLURIPOTENT STEM-CELLS | - |
dc.subject.keywordPlus | LEWY BODIES | - |
dc.subject.keywordPlus | OXIDATIVE STRESS | - |
dc.subject.keywordPlus | ANIMAL-MODELS | - |
dc.subject.keywordPlus | COMPLEX-I | - |
dc.subject.keywordPlus | HUMAN ES | - |
dc.subject.keywordPlus | DISEASE | - |
dc.subject.keywordPlus | EXPRESSION | - |
dc.subject.keywordPlus | AGGREGATION | - |
dc.subject.keywordPlus | MUTATIONS | - |
dc.subject.keywordAuthor | Parkinson's disease | - |
Items in ScholarWorks are protected by copyright, with all rights reserved, unless otherwise indicated.
(31538) 22, Soonchunhyang-ro, Asan-si, Chungcheongnam-do, Republic of Korea+82-41-530-1114
COPYRIGHT 2021 by SOONCHUNHYANG UNIVERSITY ALL RIGHTS RESERVED.
Certain data included herein are derived from the © Web of Science of Clarivate Analytics. All rights reserved.
You may not copy or re-distribute this material in whole or in part without the prior written consent of Clarivate Analytics.