A recent study published in the journal Annals of Neurology reports that healthy tissue grafted to the brain of people with Huntington’s disease, in the hope of countering this neurological disorde, also develop signs of disease several years after transplantation. This discovery has not only profound implications for our understanding of the disease and how to treat it, but could also lead to the development of new therapies against various neurodegenerative disorders.
Huntington’s disease is an inherited disease that results in neuronal degeneration causing significant motor, cognitive and psychiatric impairments gradually leading to loss of independence and death. The disease develops around the age of 40 to 50. There is no cure for this disease and current treatments only help control some symptoms without slowing the neurodegenerative process.
“Until now, we had always thought that Huntington’s disease was exclusively the result of a genetic mutation in cells, an intrinsic phenomenon that eventually led to the outbreak of the disease,” says Francesca Cicchetti, a professor at the Faculty of Medicine, Laval University, researcher at the Research Centre of the University Hospital of Quebec and first author of the study. “Our work suggests, however, that the mutated protein at the origin of the disease can also be transmitted from carrier cells to healthy cells, an unexpected discovery.”
The findings of Professor Cicchetti and her colleagues will have profound implications for the understanding of this disease and its treatment. They could also lead to the development of new therapies against other neurodegenerative diseases of the central nervous system and those related to the spread of pathological proteins, including Parkinson’s and Alzheimer’s.
In addition to Francesca Cicchetti, the authors of the study are: Steve Lacroix, Giulia Cisbani, Nicolas Vallières, Martine Saint-Pierre and Isabelle St-Amour (Research Center CHU Quebec); Diego Mantovani and Ranna Tolouei (Laboratory of Biomaterials and Bioengineering, Hôpital Saint-François d’Assise-CHU de Québec); Jeremy M Skepper (Cambridge Advanced Imaging Centre, University of Cambridge); Robert Hauser (Parkinson’s Disease and Movement Disorders Center, University of South Florida); Roger Barker (John van Geest Centre for Brain Repair, University of Cambridge); Thomas B. Freeman (Center of Excellence for Aging and Brain Repair at the University of South Florida).
Source of text: Université Laval
Photo credit: Marc Robitaille
Translation: CAN-ACN
Original Research Article:
Cicchetti F, Lacroix S, Cisbani G, Vallières N, Saint-Pierre M, St-Amour I, Tolouei R, Skepper JN, Hauser RA, Mantovani D, Barker RA, Freeman TB. Mutant huntingtin is present in neuronal grafts in huntington disease patients. Ann
Neurol. 2014 May 6. doi: 10.1002/ana.24174.
