4.4 Review

Patient-specific pluripotent stem cell-based Parkinson's disease models showing endogenous alpha-synuclein aggregation

Journal

BMB REPORTS
Volume 52, Issue 6, Pages 349-359

Publisher

KOREAN SOCIETY BIOCHEMISTRY & MOLECULAR BIOLOGY
DOI: 10.5483/BMBRep.2019.52.6.142

Keywords

Aggregation; Alpha-synuclein; Disease modeling; hiPSC; Parkinson's disease

Funding

  1. National Research Foundation of Korea (NRF) - governments of Korea (Ministry of Education) [NRF-2017R1A6A3A03010524]
  2. National Research Foundation of Korea (NRF) - governments of Korea (Ministry of Science and ICT) [NRF-2018R1C1B5045395]
  3. Hanyang University
  4. National Research Foundation of Korea [2017R1A6A3A03010524] Funding Source: Korea Institute of Science & Technology Information (KISTI), National Science & Technology Information Service (NTIS)

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After the fist research declaring the generation of human induced pluripotent stem cells (hiPSCs) in 2007, several attempts have been made to model neurodegenerative disease in vitro during the past decade. Parkinson's disease (PD) is the second most common neurodegenerative disorder, which is mainly characterized by motor dysfunction. The formation of unique and filamentous inclusion bodies called Lewy bodies (LBs) is the hallmark of both PD and dementia with LBs. The key pathology in PD is generally considered to be the alpha-synuclein (alpha-syn) accumulation, although it is still controversial whether this protein aggregation is a cause or consequence of neurodegeneration. In the present work, the recently published researches which recapitulated the alpha-syn aggregation phenomena in sporadic and familial PD hiPSC models were reviewed. Furthermore, the advantages and potentials of using patient-derived PD hiPSC with focus on alpha-syn aggregation have been discussed.

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