Creutzfeldt-Jacob disease

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... 

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. 

Two human forms of spongiform encephalopathies are kura and Creutzfeldt-Jacob disease (CJD). 

Creutzfeldt-Jacob disease—Human form of transmissible spongiform encephalopathy (TSE), a brain disease in which nerve cells of the brain are destroyed. Seen under a microscope, the brain tissue of people with TSE resembles a sponge. 

Transmissible spongiform encephalopathies (TSEs)—Brain diseases transmitted from one animal to another. Under a microscope, the brain tissue of animals and people with TSEs resembles a sponge. TSEs include variant Creutzfeldt-Jacob disease (vCJD) in humans, scrapie in sheep and goats, and bovine spongiform encephalopathy (BSE) in cows (mad cow disease). These diseases are spread by consumption of brain tissue and are thought to be caused by prions, a kind of protein. 

As mentioned in CHEC(1984), phenothiazine derivatives are best known as pharmaceuticals for the central nervous system, and are used not only for treatment of various mental illnesses <1986AXC1083> but also as neuroleptics, sedatives, analgesics, anti-emetics, and antihistamines <1987AXC1737>. A review has appeared on use of pheno-thiazines in the treatment of Creutzfeldt-Jacob disease <2001JAA411>. 

Other phenomena. Prion proteins, the mutated forms of which cause Creutzfeldt-Jacob disease, also appear to interact with metal ions. Spectra of Cu(H) bound to various mutant proteins have indicated anomalous binding behaviour.331 Hydroxyl radical generation during the interaction of mutant forms of a prion protein fragment with Fe(II) has also been reported.332... 

Lubec, G. (2002). Expression patterns of antioxidant proteins in brains of patients with sporadic Creutzfeldt-Jacob disease. Electrophoresis 23, 2541-2547. 

Previous studies have used proteome comparisons to study such neurological disorders as Alzheimer s disease (AD), Parkinson s disease (PD), frontotemporal dementia, schizophrenia, and Creutzfeldt-Jacob disease. Some examples of utilizing proteomics approaches for discovery of biomarkers for neurological disorders in human CSF are given in Table 49.2. 

Creutzfeldt-Jacob disease 14-3-3 pi otem cystadn C 2D SDS-PAGE,. SELDl-TOF and (Hairmgton et al., 1986 Sanchez... 

Ulm j Vnencak-Jones CL, Bosque P. Research on familial Creutzfeldt-Jacob Disease (FCJD) resulting in presymptomatic testing implications for the Human Genome Project. J Genet Counseling 1993 2 9-15. 

Brown, P., McShane, L.M., Zanusso, G., and Detwiler, L. 2006. Qn the question of sporadic Bovine Spongiform Encephalopathy and Creutzfeldt-Jacob Disease. Emerging Infectious Disease 12,1816-1821. 

Creutzfeldt-Jacob Disease (CJD) a. Caused by a prion protein (PrP)... 

Goldfarb, L.G., Brown, R, Mitrova, E., Cervenakova, L., Goldin, L. et al. (1991). Creutzfeldt-Jacob disease associated with the PRNP codon 200 "t mutation an analysis of 45 families. Eur. J. Epidemiol. 7, 477-486. 

Human TSEs are rare but include the Creutzfeldt-Jacob disease (CJD), Gertstmann-Straussler-Scheinker (GSS) disease, and famihal fatal insomnia. All three human diseases appear to have both familial and sporadic occurrence, and appear to be different manifestations of pathology of the same prion protein. Kuru is the human disease caused by ritual cannibalism of brain. Similarly, human vCJD appears to have originated in the mad cow BSE epidemic in the United Kingdom. The hypothesis... 

Brown, D. R., Qin, K., Herms, J. W., Madlung, A., Manson, J., Strome, R., Fraser, P. E., Kruck, T., von Bohlen, A., Schulz-Schaeffer, W., Giese, A., Westaway, D., and Kretzschmar, H. 1997. The cellular prion protein binds copper in vivo. Nature 390 684-687 Brown, R, WiU, R. G., Bradley, R., Asher, D. M., and DetwUer, L. 2001. Bovine spongiform encephalopathy and variant Creutzfeldt— Jacob disease background, evolution, and current concerns. Emerging Infect. Dis. 7 6-16... 

---