Encephalopathy

Transmissible spongiform encephalopathies (TSE) are fatal neuro-degenerative diseases of humans and animals. The underlying infectious agent, the prion, accumulates not only in the central ner-vous system (CNS) but also in secondary lymphoid organs. I will revisit the role of the immune system in peripheral prion pathogen-esis, while focusing on the mechanisms by which extraneural and extralymphatic prion infectivity develops.

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The dementia familial encephalopathy with neuroserpin inclusion bodies (FENIB) is caused by point mutations in the neuroserpin gene.We have shown a correlation between the predicted effect of the mutation and the number of intracerebral inclusions, and an inverse relationshipwith the age of onset of disease. Our previous work has shown that the intraneuronal inclusions in FENIB result from the sequential interactionbetween the reactive centre loopofone neuroserpin molecule withb-sheet A of the next. ...

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Mutations in neuroserpin anda1-antitrypsin cause these proteins to form ordered polymers that are retained within the endoplasmic reticulum of neurones and hepatocytes, respectively. The resulting inclusions underlie the dementia familial encephalopathy with neuroserpin inclusion bodies (FENIB) and Z a1-antitrypsin-associated cirrhosis.

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During pathogenesis of transmissible spongiform encephalopathies (TSEs) an abnormal form (PrP Sc ) of the host encoded prion protein (PrP C ) accu-mulates in insoluble fibrils and plaques. The two forms of PrP appear to have identical covalent structures, but differ in secondary and tertiary structure.

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Prion diseases are fatal neurodegenerative disorders caused by proteina-ceous infectious pathogens termed prions (PrP Sc ). To date, there is no pro-phylaxis or therapy available for these transmissible encephalopathies. Passive immunization with monclonal antibodies recognizing the normal host-encoded prion protein (PrP C ) has been reported to abolish PrP Sc infec-tivity and to delay onset of disease.

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Prion diseases are neurodegenerative disorders which cause Creutzfeldt– Jakob disease in humans, scrapie in sheep and bovine spongiform encephalopathy in cattle. The infectious agent is a protease resistant iso-form (PrP Sc ) of a host encoded prion protein (PrP C ). PrP Sc proteins are characterized according to size and glycoform pattern.

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The prion protein (PrP) is the key protein implicated in transmissible spongiform encephalopathies. It is a metalloprotein that binds manganese and copper. The latter is involved in the physiological function of the pro-tein. We have previously found that PrP expression inPichia pastorisaffects intracellular metal ion concentrations and that formation of protease-resistant PrP is induced by additional copper and⁄or manganese.

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Transmissible spongiform encephalopathies are fatal neurodegenerative dis-eases that are caused by unconventional pathogens and affect the central nervous system of animals and humans. Several different forms of these dis-eases result from natural infection (i.e. exposure to transmissible spongiform encephalopathy agents or prions, present in the natural environment of the respective host).

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1The epidemic of bovine spongiform encephalopathy (BSE), or ‘mad cow disease’, and the subsequent emer-gence of a new variant of Creutzfeldt–Jakob disease (vCJD) in humans, has directed great political and sci-entific attention to a family of related neurodegenera-tive protein-misfolding diseases, collectively known as transmissible spongiform encephalopathies (TSEs) or prion diseases.

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As limited structural information is available on prion protein (PrP) mis-folding and aggregation, a causative link between the specific (supra)mole-cular structure of PrP and transmissible spongiform encephalopathies remains to be elucidated.

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Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative disorders caused by an infectious agent termed a prion, which can convert normal cellular prion protein (PrP C ) into a patho-logically misfolded isoform (PrP Sc ). Taking advantage of protein misfolding cyclic amplification (PMCA), a series of experiments was conducted to investigate the possible influences of pyridine nucleotides on the propaga-

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Abnormal forms of prion protein (PrP Sc ) accumulate via structural conver-sion of normal PrP (PrP C ) in the progression of transmissible spongiform encephalopathy. Under cell-free conditions, the process can be efficiently replicated usingin vitro PrP Sc amplification methods, including protein mis-folding cyclic amplification.

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The conformational conversion of prion protein (PrP) from a native con-formation to the amyloid form is a hallmark of transmissible spongiform encephalopathies. Conversion is usually monitored by fluorescent dyes, which bind generic amyloids and are less suited for living cell imaging.

13p mobifone23 21-01-2013 39 4   Download

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Long-term tracking of neurological complications of encephalopathy and myopathy in a patient with nephropathic cystinosis: a case report and review of the literature

5p dauphong1 21-12-2011 61 5   Download

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: A reversible lesion of the corpus callosum splenium with adult influenza-associated encephalitis/encephalopathy: a case report

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Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: A patient with typical clinical features of mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) but without an obvious genetic cause: a case report

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Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Incisional hernia as an unusual cause of hepatic encephalopathy in a 62-year-old man with cirrhosis: a case repor

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Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Near fatal posterior reversible encephalopathy syndrome complicating chronic liver failure and treated by induced hypothermia and dialysis: a case report

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Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Severe hepatic encephalopathy in a patient with liver cirrhosis after administration of angiotensin-converting enzyme inhibitor/angiotensin II receptor blocker combination therapy: a case report...

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Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Hashimoto’s encephalopathy presenting with neurocognitive symptoms: a case report

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