M. W. Head and J. W. Ironside (2012) Neuropathology and Applied Neurobiology38, 296–310
Creutzfeldt–Jakob disease: prion protein type, disease phenotype and agent strain
The human transmissible spongiform encephalopathies or human prion diseases are one of the most intensively investigated groups of rare human neurodegenerative conditions. They are generally held to be unique in terms of their complex epidemiology and phenotypic variability, but they may also serve as a paradigm with which other more common protein misfolding disorders might be compared and contrasted. The clinico-pathological phenotype of human prion diseases appears to depend on a complex interaction between the prion protein genotype of the affected individual and the physico-chemical properties of the neurotoxic and transmissible agent, thought to comprise of misfolded prion protein. A major focus of research in recent years has been to define the phenotypic heterogeneity of the recognized human prion diseases, correlate this with molecular-genetic features and then determine whether this molecular-genetic classification of human prion disease defines the biological properties of the agent as determined by animal transmission studies. This review seeks to survey the field as it currently stands, summarize what has been learned, and explore what remains to be investigated in order to obtain a more complete scientific understanding of prion diseases and to protect public health.
"Familial CJD (fCJD), Gerstmann–Sträussler–Scheinker disease (GSS), fatal familial insomnia (FFI) and prion protein cerebral amyloid angiopathy (PrP-CAA) are genetic forms of human TSE. The acquired forms are transmitted from human to human, as iatrogenic CJD (iCJD) and Kuru; or from cattle to human, as variant CJD (vCJD) (Head and Ironside, 2012). In animals, relevant TSE are scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, and chronic wasting disease (CWD) in cervids (Imran and Mahmood, 2011). "
"Variant CJD is a novel human transmissible spongiform encephalopathy transmitted by the consumption of tissues from cattle affected by bovine spongiform encephalopathy (BSE) (Head & Ironside, 2012). The latter is a disease of cattle that resulted from feeding the animals with meat and bone meal. "
[Show abstract][Hide abstract] ABSTRACT: The threat of infection by conventional transfusion-transmitted agents has been essentially eliminated from the blood supply in developed countries, thus focusing attention on the potential risk from emerging infections. Over recent years, actions have been taken to manage a number of such risks to blood safety. These illustrate the inherent variability of the agents concerned and of the measures needed to define and control the risk.
British Journal of Haematology 08/2012; 159(2):135-42. DOI:10.1111/bjh.12031 · 4.71 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: With advancing age, the brain becomes increasingly susceptible to neurodegenerative diseases, most of which are characterized by the misfolding and errant aggregation of certain proteins. The induction of aggregation involves a crystallization-like seeding mechanism by which a specific protein is structurally corrupted by its misfolded conformer. The latest research indicates that, once formed, proteopathic seeds can spread from one locale to another via cellular uptake, transport, and release. Impeding this process could represent a unified therapeutic strategy for slowing the progression of a wide range of currently intractable disorders.
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