Cookies on this website

We use cookies to ensure that we give you the best experience on our website. If you click 'Accept all cookies' we'll assume that you are happy to receive all cookies and you won't see this message again. If you click 'Reject all non-essential cookies' only necessary cookies providing core functionality such as security, network management, and accessibility will be enabled. Click 'Find out more' for information on how to change your cookie settings.

The advent of induced pluripotent stem cell (iPSC)-derived neurons has revolutionized Parkinson's disease (PD) research, but single-cell transcriptomic analysis suggests unresolved cellular heterogeneity within these models. Here, we perform the largest single-cell transcriptomic study of human iPSC-derived dopaminergic neurons to elucidate gene expression dynamics in response to cytotoxic and genetic stressors. We identify multiple neuronal subtypes with transcriptionally distinct profiles and differential sensitivity to stress, highlighting cellular heterogeneity in dopamine in vitro models. We validate this disease model by showing robust expression of PD GWAS genes and overlap with postmortem adult substantia nigra neurons. Importantly, stress signatures are ameliorated using felodipine, an FDA-approved drug. Using isogenic SNCA-A53T mutants, we find perturbations in glycolysis, cholesterol metabolism, synaptic signaling, and ubiquitin-proteasomal degradation. Overall, our study reveals cell type-specific perturbations in human dopamine neurons, which will further our understanding of PD and have implications for cell replacement therapies.

Original publication

DOI

10.1016/j.celrep.2020.108263

Type

Journal article

Journal

Cell Rep

Publication Date

13/10/2020

Volume

33

Keywords

Cholesterol biosynthesis, Dopamine neurons, ER stress, Felodipine, Human iPSC, Oxidative stress, PD GWAS, Parkinson's disease, SNCA-A53T, Single-cell transcriptomics, Cell Differentiation, Cell Respiration, Cholesterol, Chromatin Assembly and Disassembly, Dopaminergic Neurons, Down-Regulation, Endoplasmic Reticulum Stress, Gene Expression Profiling, Genome-Wide Association Study, Glycolysis, Humans, Induced Pluripotent Stem Cells, Models, Biological, Oxidative Phosphorylation, Oxidative Stress, Parkinson Disease, Proteasome Endopeptidase Complex, Regression Analysis, Signal Transduction, Single-Cell Analysis, Stress, Physiological, Synapses, Transcriptome, Ubiquitin, Up-Regulation