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https://www.readbyqxmd.com/read/28741862/in-vivo-comparison-of-jellyfish-and-bovine-collagen-sponges-as-prototype-medical-devices
#1
Jonathan P Widdowson, Alex J Picton, Valerie Vince, Chris J Wright, Andrew Mearns-Spragg
Jellyfish have emerged as a source of next generation collagen that is an attractive alternative to existing sources, such as bovine and porcine, due to a plentiful supply and providing a safer source through lack of bovine spongiform encephalopathy (BSE) transmission risk and potential viral vectors, both of which could be transmitted to humans. Here we compare collagen implantable sponges derived for the first time from the Rhizostoma pulmo jellyfish. A further novelty for the research was that there was a comparison for sponges that were either uncrosslinked or crosslinked using 1-ethyl-3-(3-dimethylaminopropyl) carbodiimide hydrochloride (EDC), and an assessment on how this affected resorption, as well as their biocompatibility compared to bovine type I collagen sponges...
July 25, 2017: Journal of Biomedical Materials Research. Part B, Applied Biomaterials
https://www.readbyqxmd.com/read/28739602/loss-of-prion-protein-is-associated-with-the-development-of-insulin-resistance-and-obesity
#2
Giovanna Brito, Fernada C S Lupinacci, Flávio H Beraldo, Tiago G Santos, Martin Roffé, Marilene H Lopes, Vladmir C C de Lima, Vilma R Martins, Glaucia N M Hajj
Prion protein (PrP(C)) was initially described due to its involvement in transmissible spongiform encephalopathies. It was subsequently demonstrated to be a cell surface molecule involved in many physiological processes, such as vesicle trafficking. Herein, we investigated the roles of PrP(C) in the response to insulin and obesity development.  Two independent PrP(C) knockout (KO) and one PrP(C) overexpressing (TG20) mouse models were fed high-fat diets, and the development of insulin resistance and obesity was monitored...
July 24, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28731378/experimental-infection-of-cattle-with-a-novel-prion-derived-from-atypical-h-type-bovine-spongiform-encephalopathy
#3
Hiroyuki Okada, Kentaro Masujin, Kohtaro Miyazawa, Yoshihumi Iwamaru, Morikazu Imamura, Yuichi Matsuura, Shozo Arai, Shigeo Fukuda, Yuichi Murayama, Takashi Yokoyama
H-type bovine spongiform encephalopathy (H-BSE) is an atypical form of BSE in cattle. During passaging of H-BSE in transgenic bovinized (TgBoPrP) mice, a novel phenotype of BSE, termed BSE-SW emerged and was characterized by a short incubation time and host weight loss. To investigate the biological and biochemical properties of the BSE-SW prion, a transmission study was conducted in cattle, which were inoculated intracerebrally with brain homogenate from BSE-SW-infected TgBoPrP mice. The disease incubation period was approximately 15 months...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28721847/classical-scrapie-transmission-in-arr-arr-genotype-sheep
#4
Caroline Lacroux, Hervé Cassard, Hugh Simmons, Jean Yves Douet, Fabien Corbière, Severine Lugan, Pierette Costes, Naima Aron, Alvina Huor, Cécile Tillier, Francois Schelcher, Olivier Andreoletti
The ARR allele is considered to provide a very strong resistance against classical scrapie infection in sheep. In this study, we report the occurrence of clinical transmissible spongiform encephalopathy in ARR/ARR sheep, following their inoculation by the intracerebral route with a classical scrapie isolate. On first passage, the disease displayed an incomplete attack rate transmission, with incubation periods exceeding 6 years. On second passage, the obtained prion did not display better abilities to propagate than the original isolate...
July 18, 2017: Journal of General Virology
https://www.readbyqxmd.com/read/28714865/microglia-in-prion-diseases
#5
Adriano Aguzzi, Caihong Zhu
Prion diseases are a group of progressive and fatal neurodegenerative disorders characterized by deposition of scrapie prion protein (PrPSc) in the CNS. This deposition is accompanied by neuronal loss, spongiform change, astrogliosis, and conspicuous microglial activation. Here, we argue that microglia play an overall neuroprotective role in prion pathogenesis. Several microglia-related molecules, such as Toll-like receptors (TLRs), the complement system, cytokines, chemokines, inflammatory regulators, and phagocytosis mediators, are involved in prion pathogenesis...
July 17, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28708055/prion-disease-pathogenesis-in-the-absence-of-the-commensal-microbiota
#6
Barry M Bradford, Laura Tetlow, Neil A Mabbott
Prion diseases are a unique group of transmissible, typically sub-acute, neurodegenerative disorders. During central nervous system (CNS) prion disease, the microglia become activated and are thought to provide a protective response by scavenging and clearing prions. The mammalian intestine is host to a large burden of commensal micro-organisms, especially bacteria, termed the microbiota. The commensal microbiota has beneficial effects on host health, including through the metabolism of essential nutrients, regulation of host development and protection against pathogens...
July 15, 2017: Journal of General Virology
https://www.readbyqxmd.com/read/28708047/temporal-patterns-of-chronic-wasting-disease-prion-excretion-in-three-cervid-species
#7
Ian H Plummer, Scott D Wright, Chad J Johnson, Joel A Pedersen, Michael D Samuel
Chronic wasting disease (CWD) is the only naturally occurring transmissible spongiform encephalopathy affecting free-ranging wildlife populations. Transmission of CWD occurs by direct contact or through contaminated environments; however, little is known about the temporal patterns of CWD prion excretion and shedding in wild cervids. We tested the urine and faeces of three species of captive cervids (elk, mule and white-tailed deer) at 6, 12, 18 and 24 months after oral inoculation to evaluate the temporal, species- and genotype-specific factors affecting the excretion of CWD prions...
July 15, 2017: Journal of General Virology
https://www.readbyqxmd.com/read/28707717/an-autopsied-case-of-corticobasal-degeneration-presenting-with-frontotemporal-dementia-followed-by-myoclonus
#8
Yasushi Iwasaki, Keiko Mori, Masumi Ito, Maya Mimuro, Mari Yoshida
A Japanese woman developed frontotemporal dementia (FTD)-like symptoms of abnormal behavior, such as stereotyped behavior and disinhibition. The patient developed these symptoms at the age of 59 years, although aphasia symptoms were not apparent at early disease stages. Progressive parkinsonism was dominant on the left side, and conspicuous myoclonus was recognized in the late disease stage. MRI indicated severe, right side-dominant frontotemporal lobe atrophy with white matter degeneration. Brainstem and cerebellar atrophy were also observed...
July 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28680785/a-case-report-of-probable-sporadic-creutzfeldt-jakob-disease-how-to-approach-early-diagnosis
#9
Bowei Tan, Carlos Morales Mangual, Iftekhar Mahmud, Nosakhare D Tongo, Larisa Mararenko, Arthur Kay
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. The rarity of this disease and varied initial symptoms make the early diagnosis fairly challenging. Here, we present a case initially admitted for confusion and bizarre behaviors. She had acute deterioration of mental status, akinetic mutism, and myoclonus jerks four weeks later. Cerebrospinal fluid (CSF) analysis was positive for protein 14-3-3. Brain magnetic resonance imaging (MRI) showed hyperintensities in the bilateral cortex, basal ganglia, and thalami in diffusion-weighted imaging (DWI)...
May 30, 2017: Curēus
https://www.readbyqxmd.com/read/28674232/a-cross-sectional-study-of-prnp-gene-in-two-native-sicilian-goat-populations-in-italy-a-relation-between-prion-gene-polymorphisms-and-scrapie-incidence
#10
Sergio Migliore, Stefano Agnello, Salvatore D'Avola, Wilfred Goldmann, Vincenzo Di Marco Lo Presti, Maria Vitale
Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative diseases affecting humans and animals, and scrapie in small ruminants is considered the archetype of TSEs. Derivata di Siria is a native dairy goat of Sicily (south Italy), which is related to Syrian goat breeds. Scrapie disease is considered endemic in Sicily since 1997, following the administration of an infected vaccine.Derivata di Siria goatswere involved in six of 66 scrapie-infected flocks in Sicily. Prion protein gene (PRNP) analysis revealed that none of the scrapie cases carried the p...
June 2017: Journal of Genetics
https://www.readbyqxmd.com/read/28670273/types-and-strains-their-essential-role-in-understanding-protein-aggregation-in-neurodegenerative-diseases
#11
REVIEW
Wiebke M Wemheuer, Arne Wrede, Walter J Schulz-Schaeffer
Protein misfolding and aggregation is a key event in diseases like Alzheimer's disease (AD) or Parkinson's disease (PD) and is associated with neurodegeneration. Factors that initiate protein misfolding and the role of protein aggregation in the pathophysiology of disease pose major challenges to the neuroscientific community. Interestingly, although the accumulation of the same misfolded protein, e.g., α-synuclein is detectable in all idiopathic PD patients, the disease spectrum covers a variety of different clinical presentations and disease courses...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28644254/sporadic-creutzfeldt-jakob-disease-with-unilateral-symptoms-in-the-setting-of-metastatic-renal-cell-carcinoma
#12
Kyle C Rossi, Christine M Stahl, Pengfei Zhang, John W Liang, Lara V Marcuse, Fred Lublin
INTRODUCTION: Although it is not rare for magnetic resonance imaging findings in Creutzfeldt-Jakob disease to be asymmetric, unilateral clinical syndromes are uncommonly reported and may confound diagnosis. In addition, neurological paraneoplastic syndromes are not common in renal cell carcinoma, though there are cases reported, often without an offending antibody isolated. CASE REPORT: A 66-year-old man was admitted with 1 month of left-sided numbness and "loss of control" of the left arm...
July 2017: Neurologist
https://www.readbyqxmd.com/read/28630454/treatment-with-a-non-toxic-self-replicating-anti-prion-delays-or-prevents-prion-disease-in-vivo
#13
R Diaz-Espinoza, R Morales, L Concha-Marambio, I Moreno-Gonzalez, F Moda, C Soto
Transmissible spongiform encephalopathies (TSEs) are fatal neurological disorders caused by prions, which are composed of a misfolded protein (PrP(Sc)) that self-propagates in the brain of infected individuals by converting the normal prion protein (PrP(C)) into the pathological isoform. Here, we report a novel experimental strategy for preventing prion disease based on producing a self-replicating, but innocuous PrP(Sc)-like form, termed anti-prion, which can compete with the replication of pathogenic prions...
June 20, 2017: Molecular Psychiatry
https://www.readbyqxmd.com/read/28625169/is-there-a-decline-in-bovine-spongiform-encephalopathy-cases-born-after-reinforced-feed-bans-a-modelling-study-in-eu-member-states
#14
M E Arnold, R R L Simons, J Hope, N Gibbens, A L Adkin
Occasional cases of classical bovine spongiform encephalopathy (BSE) still continue to occur within the European Union (EU) for animals born after reinforced feed bans (BARBs), which should in theory have eliminated all risk of infection. The study aimed to determine (i) whether a common rate of decline of BSE infection was evident across EU member states, i.e. to determine whether control measures have been equally effective in all member states, (ii) whether there was any evidence of spontaneous occurrence of BSE in the data and (iii) the expected date for the last BSE case in UK...
June 19, 2017: Epidemiology and Infection
https://www.readbyqxmd.com/read/28596963/prion-diagnosis-application-of-real-time-quaking-induced-conversion
#15
REVIEW
Hae-Eun Kang, Youngwon Mo, Raihah Abd Rahim, Hye-Mi Lee, Chongsuk Ryou
Prions composed of pathogenic scrapie prion protein (PrP(Sc)) are infectious pathogens that cause progressive neurological conditions known as prion diseases or transmissible spongiform encephalopathies. Although these diseases pose considerable risk to public health, procedures for early diagnosis have not been established. One of the most recent attempts at sensitive and specific detection of prions is the real-time quaking-induced conversion (RT-QuIC) method, which measures the activity of PrP(Sc) aggregates or amyloid formation triggered by PrP(Sc) seeds in the presence of recombinant PrP...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28568896/an-autopsied-case-of-mv2k%C3%A2-%C3%A2-c-type-sporadic-creutzfeldt-jakob-disease-presenting-with-widespread-cerebral-cortical-involvement-and-kuru-plaques
#16
Yasushi Iwasaki, Yufuko Saito, Ikuko Aiba, Atsushi Kobayashi, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida
MV2-type sporadic Creutzfeldt-Jakob disease (sCJD), which was previously called "Kuru-plaque variant", was gradually revealed to have a wide spectrum and has been classified into three pathological subtypes: MV2K, MV2C and MV2K + C. We herein describe the detailed clinical findings and neuropathologic observations from an autopsied MV2K + C-type Japanese sCJD case with widespread cerebral cortical pathology and Kuru plaques. In the early stages of the disease, the patient exhibited gait disturbance with ataxia and dysarthria as well as gradual appearance of cognitive dysfunction...
June 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28549449/fatal-familial-insomnia-with-abnormal-signals-on-routine-mri-a-case-report-and-literature-review
#17
Tingting Lu, Yuhang Pan, Lisheng Peng, Feng Qin, Xiaobo Sun, Zhengqi Lu, Wei Qiu
BACKGROUND: Fatal familial insomnia (FFI) is a rare autosomal dominant disease caused by the PRNP D178N/129 M mutation. Routine brain CT and MRI usually reveal non-specific features. We report a patient with FFI presenting with diffuse abnormal signals on MRI, later confirmed as combined with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). CASE PRESENTATION: The patient was a 58-year-old female, whose main clinical manifestations were insomnia, movement disorders, autonomic hyperactivity and mental deterioration...
May 26, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28549342/mgr1-antigen-37-kda-laminin-receptor-precursor-promotes-cellular-prion-protein-induced-multi-drug-resistance-of-gastric-cancer
#18
Guanhong Luo, Weijie Wang, Qiong Wu, Yuanyuan Lu, Tao Su, Nan Gu, Kai Li, Jingbo Wang, Rui Du, Xiaodi Zhao, Xiaohua Li, Rui Fan, Hongbo Zhang, Yongzhan Nie, Xinmin Zhou, Yongquan Shi, Jie Liang, Xin Wang, Daiming Fan
Cellular prion protein (PrPC), the infective agent of transmissible spongiform encephalopathies, is thought to be related to several cellular physiological and physiopathological processes. We have previously reported that PrPC participates in multi-drug-resistance of gastric cancer. As the salient ligand molecule of PrP for participating in internalization and propagation of the scrapie form of prion protein (PrPSc), 37 kDa laminin receptor precursor protein (37LRP) shared the same gene coding sequence of MGr1-Ag, another protein previously found to be involved in multi-drug-resistance of gastric cancer in our lab...
May 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28545018/fixed-differences-in-the-3-utr-of-buffalo-prnp-gene-provide-binding-sites-for-mirnas-post-transcriptional-regulation
#19
Hui Zhao, Siqi Wang, Lixia Guo, Yanli Du, Linlin Liu, Tengfei Ma, Newton O Otecko, Canpeng Li, Yaping Zhang
Bovine spongiform encephalopathy, a member of transmissible spongiform encephalopathies, has not been reported in buffaloes, Bubalus bubalis. Prion protein (PrP), encoded by the prion protein gene (PRNP), is fundamental in the pathogenesis of transmissible spongiform encephalopathies. We previously showed that buffaloes express more PrP proteins but lower PRNP mRNA than cattle in several pivotal tissues like the obex. Therefore, we sought to establish whether genetic variability in PRNP 3'UTR, mediated by miRNA down-regulation, causes PrP expression differences between cattle and buffaloes...
July 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28539446/infectious-prions-in-the-pregnancy-microenvironment-of-cwd-infected-reeves-muntjac-deer
#20
Amy V Nalls, Erin McNulty, Clare E Hoover, Laura A Pulscher, Edward A Hoover, Candace K Mathiason
Ample evidence exists for the presence of infectious agents at the maternal-fetal interface, often with grave outcomes to the developing fetus (i.e. zika virus, brucella, cytomegalovirus, toxoplasma). While less studied, pregnancy-related transmissible spongiform encephalopathies (TSEs) have been implicated in several species, including humans. Our previous work has shown that prions can be transferred from mother-to-offspring resulting in the development of clinical TSE disease in offspring born to CWD-infected muntjac dams (64)...
May 24, 2017: Journal of Virology
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