5 articles for “Prion Disease”

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Case ReportOpen accessIn the keywordsBrief Report: 20-Year Follow-Up of an Infant with Neurological Impairment Born to a Mother with Variant Creutzfeldt-Jakob DiseaseJustin Penner, Anjali Rampersad, James E. Burns et al.A term infant born via uncomplicated caesarian section was noted to be jittery, hypotonic, and have pooling of secretions at birth. Over the first four weeks of life, the infant developed four limb hypertonia, hyperreflexia, and clonus. MRI at 3.5 weeks old showed brain immaturity, poor myelination, and increased signal in the dorsal thalami. At six months, dystonic features with … …American Journal of Infectious Diseases, Vol. 21, Issue 1 · 7 Mar 2025 · 7,660 accessesView PDF Cite

Case ReportOpen accessIn the keywordsA Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler VelvetJoseph Wiedemer, Yanely Sanchez Ceja, An Cao, Ibrahim MustafaCreutzfeldt-Jakob Disease (CJD) is a rare rapidly progressive neurodegenerative prion disorder that is usually sporadic but may also be acquired from exposure to infected sources, classically via infections of bovine or human etiology. Cervid transmission of CJD is of particular concern in North America given the rapid spread of Chronic Wasting Disease (CWD)- the Cervid version of CJD. We present … …American Journal of Infectious Diseases, Vol. 17, Issue 1 · 18 Mar 2021 · 7,551 accessesView PDF Cite

Research ArticleOpen accessIn the keywordsPROBABLE CREUTZFELDT JAKOB DISEASE: CASE REPORTYanes Brum Bello, Alexandre Cossenza Pettezzoni De Almeida, Daniela Mendonca Sueth et al.Prion diseases are associated with the accumulation of an abnormal isoformof cellular prion protein (PrPSc), which is the principal constituent of prions. Prions replicate in lymphoreticular tissues before neuroinvasion, suggesting that lymphoreticular biopsy samples may allow early diagnosis by detection of PrPSc. Creutzfeldt-Jakob Disease (CJD) is the most frequent prion disease in humans. Clinical diagnosis of sporadic (CJD) is based … …Current Research in Medicine, Vol. 5, Issue 1 · 14 Mar 2014 · 8,133 accessesView PDF Cite

Research ArticleOpen accessIn the keywordsCholesterol Metabolism in Brain and Skin Fibroblasts from Sarda Breed Sheep With Scrapie-resistant and Scrapie-susceptible GenotypesAlessandra Pani, Claudia Abete, Claudia Norfo et al.Scrapie is a fatal spongiform encephalopathy of sheep, a transmissible form of prion disease caused by neuronal accumulation of the aberrantly conformed prion protein (PrPsc). Currently, no ante-mortem diagnostic tests are available to detect this untreatable disease in the pre-clinical stage, thus making difficult to control its spread. Recent evidence suggests that the production of PrPsc can be modulated by … …American Journal of Infectious Diseases, Vol. 3, Issue 3 · 30 Sep 2007 · 7,257 accessesView PDF Cite

Research ArticleOpen accessIn the abstractIntrinsically Unstructured Proteins: Potential Targets for Drug DiscoveryPathan Salma, Chintan Chhatbar, Sriram SeshadriProblem statement: The function of a protein is dependent on its three-dimensional structure. However, numerous proteins lacking intrinsic globular 3D structure under physiological conditions had been recognized. These proteins are frequently involved in some of the most critical cellular control mechanisms and it appears that their rapid turnover, aided by their unstructured nature in the unbound state, provides a level … …American Journal of Infectious Diseases, Vol. 5, Issue 2 · 30 Jun 2009 · 7,578 accessesView PDF Cite