Creutzfeldt

The transmissible spongiform encephalopathies, or prion diseases, are fatal neurodegenerative diseases characterized by spongiform changes, astrocytic gliomas, and neuronal loss resulting from the deposition of insoluble protein aggregates in neural cells. They include Creutzfeldt-Jakob disease in humans, scrapie in... 

Prions—protein particles that lack nucleic acid— cause fatal transmissible spongiform encephalopathies such as Creutzfeldt-Jakob disease, scrapie, and bovine spongiform encephalopathy. Prion diseases involve an altered secondary-tertiary strucmre of a namrally occurring protein, PrPc. When PrPc interacts with its pathologic isoform PrPSc, its conformation is transformed from a predominantly a-helical strucmre to the P-sheet strucmre characteristic of PrPSc. 

Other disorders in which glycoproteins have been implicated include hepatitis B and C, Creutzfeldt-Jakob disease, and diarrheas due to a number of bacterial enterotoxins. It is hoped that basic smdies of glycoproteins and other glycoconjugates (ie, the field of glycobiology) will lead to effective treatments for diseases in which these molecules are involved. Already, at least two disorders have been found to respond to oral supplements of sugars. 

Microorganisms surviving M. tuberculosis Bacterial spores HBV and prions as in Creutzfeldt-Jakob disease Bacterial spores Prions Extreme challenge of resistant bacterial spores Prions (insufficient data)... 

If the source of the infechon persists, after onset, then the incidence of new cases is maintained at a level which is commensurate with the infechvity of the pathogen and the ffequeney of exposure of individuals. In this manner, if cases of the variant Creutzfeldt-Jacob disease (vCJD), first recognized in the mid-1990s, relates to human exposure to bovine spongiform encephalopathy-infected beef in the early 1980s, then... 

Uncomplicated, with delirium, with delusions, and with depressed mood Dementia due to HIV disease Dementia due to head trauma Dementia due to Parkinson s disease Dementia due to Huntington s disease Dementia due to Pick s disease Dementia due to Creutzfeldt-Jakob disease Dementia due to a specific general medical condition (specify) Dementia that is substance-induced Dementia due to multiple etiologies Dementia not otherwise specified... 

P. Brown, C. C. Gajdusek, C. J. Gibbs, and D. N. Asher, Potential epidemic of Creutzfeldt-Jakob disease from human growth hormone therapy, N. Engl. J. Med., 313(12), 728 (1985). 

Creutzfeldt-Jakob disease (CJD) New variant CJD Gerstmann-Straussler-Scheinker disease Fatal familial insomnia Kuru Prion protein Extracellular deposits... 

Human prion disease models have also been developed in mice [154,155]. Crossing the species barrier into an experimentally accessible animal system, the prions responsible for Creutzfeldt Jakob disease, new variant CJD, Gerstmann-Straussler-Scheinker disease, and fatal familial insomnia produce a reproducible time-dependent neuronal degeneration leading to death. 

The conformational plasticity supported by mobile regions within native proteins, partially denatured protein states such as molten globules, and natively unfolded proteins underlies many of the conformational (protein misfolding) diseases (Carrell and Lomas, 1997 Dobson et al., 2001). Many of these diseases involve amyloid fibril formation, as in amyloidosis from mutant human lysozymes, neurodegenerative diseases such as Parkinson s and Alzheimer s due to the hbrillogenic propensities of a -synuclein and tau, and the prion encephalopathies such as scrapie, BSE, and new variant Creutzfeldt-Jacob disease (CJD) where amyloid fibril formation is triggered by exposure to the amyloid form of the prion protein. In addition, aggregation of serine protease inhibitors such as a j-antitrypsin is responsible for diseases such as emphysema and cirrhosis. 

Suggested Alternatives for Differential Diagnosis Encephalitis, Herpes Simplex, tetanus, Guillain-Barre syndrome, poliomyelitis, transverse myelitis, cerebrovascular accident, psychosis, intracranial mass, epilepsy, atropine poisoning, and Creutzfeldt-Jacob disease. 

It overcomes problems of product safety. Direct extraction of product from some native biological sources has, in the past, led to the unwitting transmission of disease. Examples include the transmission of blood-borne pathogens such as hepatitis B and C and human immunodeficiency virus (HIV) via infected blood products and the transmission of Creutzfeldt-Jakob disease to persons receiving human growth hormone (GH) preparations derived from human pituitaries. 

CREUTZFELDT-JAKOB DISEASE AND OTHER PRION DISEASES 662... 

Palmer, M. S., Dryden, A. J., Hughes, J. T. and Collinge, J. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 352 340-342,1991. 

Pocchiari, M., Puopolo, M., Croes, E. A. et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 127 2348-2359,2004. 

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

Brown, P. et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 55 1075-1081, 2000. 

Collinge J. Variant Creutzfeldt-Jakob disease. Lancet 354 317-323,1999. 

CNTF ciliary neurotrophic factor fCJD familial Creutzfeldt-Jakob disease... 

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