NR ADOV
AU Dormont,D.
TI [Natural history of human transmissible subacute spongiform encephalopathies]
OT Histoire naturelle des encephalopathies subaigues spongiformes transmissibles humaines
QU Transfusion Clinique et Biologique 1994; 1(5): 319-31
PT journal article; review; review, tutorial
AB Transmissible spongiform subacute encephalopathies are rare fatal diseases which comprise in humans Creutzfeldt-Jakob disease (CJD), Kuru, Gerstmann-Sträussler Scheinker, and Fatal Familial Insomnia (FFI). Their etiologic agents (Prions or TSA, for transmissible spongiform encephalopathy agents) are still unknown. TSA/prions resist all the physico-chemical procedures which are efficient against the other micro-organisms. These diseases are characterised by a long incubation period which may be as long as 40 years. Clinically, symptoms are only neurological, without any sign of immune response either in blood or cerebrospinal fluid. Neuropothalogy includes neuronal vacuolisation, neuronal death, spongiosis, gliosis with hyperastrocytosis. The biochemical hallmark is the post-translational accumulation of a host-encoded protein, the prion protein (PrP). In infected individuals, PrP accumulates under a proteinase K resistant isoform (PrPres) which amino acid sequence does not differ from the normal isoform (PrP-c) PrP gene (PRNP) is located on chromosome 20 in humans, and is the major determinant of the susceptibility to TSA/prions. Several hypotheses have been raised to explain the uncommon biologic properties of these agents. The prion hypothesis postulates that the agent is only composed of proteins, mainly the PrPres. Others support the presence of a host independent genetic information of which PrP could be the virulence factor.
ZR 80
MH Adult; Animal; Blood Transfusion/adverse effects; Central Nervous System/pathology/virology; Child, Preschool; Disease Models, Animal; English Abstract; Hamsters; Human; Infant; Lentivirus/genetics/pathogenicity; Mice; Models, Biological; *Prion Diseases/epidemiology/pathology/transmission/virology; Prions/chemistry/genetics/isolation & purification; Transplantation/adverse effects
AD Laboratoire de Neuropathologie experimentale et Neurovirologie, Centre de Recherches du Service de Sante des Armees, Fontenay-aux-Roses.
SP französisch
PO Frankreich