Abstract：
:There is widespread transcriptional dysregulation in Huntington's disease (HD) brain, but analysis is inevitably limited by advanced disease and postmortem changes. However, mutant HTT is ubiquitously expressed and acts systemically, meaning blood, which is readily available and contains cells that are dysfunctional in HD, could act as a surrogate for brain tissue. We conducted an RNA-Seq transcriptomic analysis using whole blood from two HD cohorts, and performed gene set enrichment analysis using public databases and weighted correlation network analysis modules from HD and control brain datasets. We identified dysregulated gene sets in blood that replicated in the independent cohorts, correlated with disease severity, corresponded to the most significantly dysregulated modules in the HD caudate, the most prominently affected brain region, and significantly overlapped with the transcriptional signature of HD myeloid cells. High-throughput sequencing technologies and use of gene sets likely surmounted the limitations of previously inconsistent HD blood expression studies. Our results suggest transcription is disrupted in peripheral cells in HD through mechanisms that parallel those in brain. Immune upregulation in HD overlapped with Alzheimer's disease, suggesting a common pathogenic mechanism involving macrophage phagocytosis and microglial synaptic pruning, and raises the potential for shared therapeutic approaches.
journal_name
Sci Repjournal_title
Scientific reportsauthors
Hensman Moss DJ,Flower MD,Lo KK,Miller JR,van Ommen GB,'t Hoen PA,Stone TC,Guinee A,Langbehn DR,Jones L,Plagnol V,van Roon-Mom WM,Holmans P,Tabrizi SJdoi
10.1038/srep44849subject
Has Abstractpub_date
2017-03-21 00:00:00pages
44849issn
2045-2322pii
srep44849journal_volume
7pub_type
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