Nature Of Neurodegenerative Diseases Research Articles

Measuring Illness beliefs in neurodegenerative disease: Why we need to be specific

Positive perceptions of illness are typically associated with good health outcomes. However, this may not be true for all domains of illness perception in neurodegenerative diseases because of their progressive incurable nature. The appropriateness of current measures of illness belief in these conditions is not known. The validity and reliability of the Illness Perception Questionnaire-Revised was evaluated in 215 participants with Parkinson's disease. A confirmatory factor analysis supported the structure of the Illness Perception Questionnaire-Revised with the exception of the treatment control domain. It is important to consider the nature of neurodegenerative diseases and limits of symptom control when planning interventions.

Spreading proteins in neurodegeneration: where do they take us?

The ‘prion-like’ nature of neurodegenerative diseases has been mooted for a number of years. The central concept is of self-propagating misfolded proteins spreading from neuron to neuron, associated with more or less stereotypical patterns of neurodegeneration in specific diseases. Apart from prion protein (PrP) in the archetypal prion diseases, the evidence that amyloid-β, tau, and now—in this issue of Brain —α-synuclein, all propagate through the brain is compelling, with implications for the pathogenesis of Alzheimer’s disease, frontotemporal dementias and other tauopathies, and Parkinson’s disease. The spread of neurodegenerative diseases through protein misfolding thus appears to be a truly generic phenomenon. Why does this matter and where does it take us? The inescapable fact of neurodegenerative diseases is that we currently have no effective way of treating them. However, the more we learn about pathogenesis, the more likely we are to identify therapeutic approaches that could succeed. So how does the spreading protein hypothesis help us? Will inhibiting cell-to-cell transmission of these pathological conformers prevent neurodegeneration? Importantly, what is the connection between spread of proteins and actual neurodegeneration? The study by Masuda-Suzukake et al. (2013) goes beyond confirming the place of α-synuclein on the list of self-propagating proteins. It raises interesting questions about the link—or lack of it—between transmission of pathological proteins and their neurotoxic effects. The relationship between toxicity and ‘infectivity’ is an important phenomenon in prion and other protein misfolding disorders. Increasing our insights into this is likely to inform our approach to understanding and treating these diseases. …

Good riddance to bad rubbish

Autophagy — cellular ‘self-eating’ — can be induced by stress, but it also acts continuously in a housekeeping role, disposing of unwanted proteins. Can it protect against neurodegenerative diseases? Two papers this week suggest that the process of protein degradation and clearance of cellular components may be more important in maintaining the health of the nervous system than was thought. Both groups show that inhibition of autophagy in mouse brain cells results in neurodegeneration and early death. Autophagy, the protein degradation and recycling of cellular components, is important for the normal growth and development of a cell. The finding that the continual clearance of cellular components is essential for maintaining neuronal health should open up new avenues of research into the nature of neurodegenerative diseases.