Parkinson’s disease: what the model systems have taught us so far

Journal of genetics

NIMA DOLATABADI^1,2¹  DORIT TRUDLER^1,2²  SWAGATA GHATAK^1,2³ 

[1] Allele Biotechnology and Pharmaceuticals, San Diego, CA 92121, USA^3;Departments of Molecular Medicine and Neuroscience, and Neuroscience Translational Center, The Scripps Research Institute, La Jolla, CA 92037, USA^2;Neurodegenerative Disease Center, Scintillon Institute, San Diego, CA 92121, USA^1

关键词: Parkinson’s disease;    neurodegenerative disease;    cell reprogramming;    stem cells;   

DOI  :  

学科分类：生物科学（综合）

来源: Indian Academy of Sciences

【 摘 要 】

Parkinson’s disease (PD) is a debilitating neurodegenerative disorder, for which people above the age of 60 show an increased risk. Although there has been great advancement in understanding the disease-related abnormalities in brain circuitry and development of symptomatic treatments, a cure for PD remains elusive. The discovery of PD associated gene mutations and environmental toxins have yielded animal models of the disease. These models could recapitulate several key aspects of PD, and provide more insights into the disease pathogenesis. They have also revealed novel aspects of the disease mechanism including noncell autonomous events and spreading of pathogenic protein species across the brain. Nevertheless, none of these models so far can comprehensively represent all aspects of the human disease. While the field is still searching for the perfect model system, recent developments in stem cell biology have provided a new dimension to modelling PD, especially doing it in a patient-specific manner. In the current review, we attempt to summarize the key findings in the areas discussed above, and highlight how the core PD pathologydistinguishes itself from other neurodegenerative disorders while also resembling them in many aspects.

【 授权许可】

CC BY