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To create a Parkinson’s disease-specific cell line which could be used as a model to gain a better understanding of the condition and investigate treatments.

Skin cells from a patient with an inherited form of Parkinson’s disease were used to create induced pluripotent stem (IPS) cells, which were then differentiated to dopaminergic (DA) neurons. The characteristics of these cells were compared with DA neurons created from IPS cells derived from a healthy adult and embryonic stem cells. They also compared the sensitivity of these cell lines to oxidative stress, mitochondrial impairment and impaired protein degradation, all of which have been implicated in PD.

The DA neurons derived from the patient with PD retained central features of the disease such as accumulation of a- synuclein protein and upregulation of oxidative stress response genes. In addition, when compared to unaffected neurons they were also slightly more sensitive to factors that induced oxidative stress or caused impairment of mitochondrial function or protein degradation.

The authors conclude that the experiments demonstrate the usefulness of IPS cells in studying PD, but point out a number of limitations in such experiments that can slow progress,  including difficulties in production of large numbers of these cells and isolation of solely DA neurons. In addition, the environmental and genetic factors that trigger their neurodegeneration in PD are not fully understood. They conclude that such systems may be better employed to address specific questions.

This is a proof of principle study demonstrating that cells from a person with a monogenic mutation can be used to produce IPS cells and subsequently neurons exhibiting PD phenotypes that may be related back to the mutation. This opens the door for further work investigating how different monogenic mutations are involved in PD, thereby leading to a better understanding of the disease and possible targets for therapy. However, this study also indicates that different methods of growing cells may affect experimental results.