Disease Creutzfeldt-Jakob

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)... 

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. 

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... 

Creutzfeldt-Jakob disease (CJD) Alzheimer s disease (AD) Hemodialysis-related amyloidosis Primary systemic amyloidosis Secondary systemic amyloidosis Familial amyloid polyneuropathy I Familial amyloid polyneuropathy III Cerebral amyloid angiopathy Finnish hereditary systemic amyloidosis Type II diabetes Injection-localized amyloidosis Medullary thyroid carcinoma Atrial amyloidosis... 

Giaccone, G., Canciani, B., Puoti, G., Rossi, G., Goffredo, D., Iussich, S., Fociani, P., Tagliavini, F., and Bugiani, O. (2000). Creutzfeldt-Jakob disease Carnoy s fixative improves the immunohistochemistry of the proteinase K-resistant prion protein. Brain Pathol. 10, 31-37. 

The normal cellular form of prion protein (PrPc) can exist as a Cu-metalloprotein in vivo (492). This PrPc is a precursor of the pathogenic protease-resistant form PrPsc, which is thought to cause scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt—Jakob disease. Two octa-repeats of PHGGGWGQ have been proposed as Cu(II) binding sites centered on histidine (493). They lack secondary and tertiary structure in the absence of Cu(II). Neurons may therefore have special mechanisms to regulate the distribution of copper. 

Bovine spongiform encephalopathy (BSE or mad cow disease) is a progressive neurological degenerative disease in cattle. It is caused by a mutated protein called a prion. BSE was first reported in the United Kingdom in 1986. Creutzfeldt-Jakob disease (CJD) is a rare disease that occurs in humans. Evidence to date indicates it is possible for humans to acquire CJD after consuming BSE-contaminated cattle products. 

Partanen, J. (2003). Genetic susceptibility to variant Creutzfeldt-Jakob disease, Lancet, 361, 447. 

With the background of the mad cow crisis in Europe, questions relating to the prion diseases have attracted intensive interest. It is now widely accepted that prion diseases, such as Creutzfeldt-Jakob disease (CJd) in humans and bovine spongiform encephalopathy (BSE) are caused by a conformational change of the prion protein (PrP) from a normally folded cellular form, PrP ", to an alternate, aggregation-prone, pathogenic scrapie form,... 

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