An inter-domain regulatory mechanism controls toxic activities of PrPC.

abstract：:Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases. They can be sporadic, genetic, or infectious disorders involving post-translational modifications of the cellular prion protein (PrP(C)). Prions (PrP(Sc)) are characterized by their infectious property and intrinsic ability to conver...

journal_title：Prion

authors： Benetti F,Legname G

更新日期：2009-10-01 00:00:00

abstract：:The prion paradigm is increasingly invoked to explain the molecular pathogenesis of neurodegenerative diseases involving the misfolding and aggregation of proteins other than the prion protein (PrP). Extensive evidence from in vitro and in vivo studies indicates that misfolded and aggregated Aβ peptide, which is the p...

journal_title：Prion

authors： Rasmussen J,Jucker M,Walker LC

更新日期：2017-07-04 00:00:00

abstract：:Conversion of native cellular prion protein (PrPc) from an α-helical structure to a toxic and infectious β-sheet structure (PrPSc) is a critical step in the development of prion disease. There are some indications that the formation of PrPSc is preceded by a β-sheet rich PrP (PrPβ) form which is non-infectious, but is...

journal_title：Prion

authors： Ladner-Keay CL,LeVatte M,Wishart DS

更新日期：2016-11-01 00:00:00

abstract：:Genetic prion diseases are degenerative brain disorders caused by mutations in the gene encoding the prion protein (PrP). Different PrP mutations cause different diseases, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome and fatal familial insomnia (FFI). The reason for this var...

journal_title：Prion

authors： Chiesa R,Restelli E,Comerio L,Del Gallo F,Imeri L

更新日期：2016-03-03 00:00:00

abstract：:The degeneration of pre-synaptic boutons in the stratum radiatum of the dorsal hippocampus is one of earliest components of neurodegeneration in several models of murine prion disease. We recently showed that blockade of synaptic transmission by infusion of botulinum neurotoxin A (BoNT/A) into the hippocampus several ...

journal_title：Prion

authors： Caleo M,Restani L,Perry VH

更新日期：2013-03-01 00:00:00

abstract：:Many neurodegenerative disorders share common features including the accumulation of aggregated misfolded proteins, neuroinflammation and the induction of apoptosis. While the contributions of each of these individual elements to neuronal death remain unclear, a commonly used antibiotic, minocycline, has been shown to...

journal_title：Prion

authors： Noble W,Garwood CJ,Hanger DP

更新日期：2009-04-01 00:00:00

abstract：:Prions are self-propagating infectious protein aggregates of mammals and fungi. The exact mechanism of prion formation is poorly understood. In a recent study, a comparative analysis of the aggregation propensities of chimeric proteins derived from the yeast Sup35p and mouse PrP prion proteins was performed in neurobl...

journal_title：Prion

authors： Krammer C,Kremmer E,Schätzl HM,Vorberg I

更新日期：2008-07-01 00:00:00

abstract：:Prion diseases are subacute neurodegenerative diseases that affect humans and a range of domestic and free-ranging animal species. These diseases are characterized by the accumulation of PrP (Sc), an abnormally folded isoform of the cellular prion protein (PrP (C)), in affected tissues. The pathology during prion dise...

journal_title：Prion

authors： Mabbott NA

更新日期：2012-09-01 00:00:00

abstract：:Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative diseases caused by the misfolding of the cellular prion protein to an infectious form PrP(Sc). The intercellular transfer of PrP(Sc) is a question of immediate interest as the cell-to-cell movement of the infectious particle causes the i...

journal_title：Prion

authors： Zhu S,Victoria GS,Marzo L,Ghosh R,Zurzolo C

更新日期：2015-01-01 00:00:00

abstract：:Growing evidence indicates that astrocytes cannot be just considered as passive supportive cells deputed to preserve neuronal activity and survival, but rather they are involved in a striking number of active functions that are critical to the performance of the central nervous system (CNS). As a consequence, it is be...

journal_title：Prion

authors： Brambilla L,Martorana F,Rossi D

更新日期：2013-01-01 00:00:00

abstract：:The yeast prion [PSI(+)] represents an aggregated state of the translational release factor Sup35 (eRF3) and deprives termination complexes of functional Sup35, resulting in nonsense codon suppression. Protein-remodeling factor Hsp104 is involved in thermotolerance and [PSI(+)] propagation, however the structure-and-f...

journal_title：Prion

authors： Takahashi A,Hara H,Kurahashi H,Nakamura Y

更新日期：2007-01-01 00:00:00

abstract：:Shadoo (Sho) is a brain glycoprotein with similarities to the unstructured region of PrP (C) . Frameshift alleles of the Sho gene, Sprn, are reported in variant Creutzfeldt-Jakob disease (vCJD) patients while Sprn mRNA knockdown in PrP-null (Prnp(0/0) ) embryos produces lethality, advancing Sho as the hypothetical PrP...

journal_title：Prion

authors： Daude N,Westaway D

更新日期：2012-11-01 00:00:00

abstract：:Perturbations of calcium homeostasis have been associated with several neurodegenerative disorders. A common polymorphism (rs2986017) in the CALHM1 gene, coding for a regulator of calcium homeostasis, is a genetic risk factor for the development of Alzheimer disease (AD). Although some authors failed to confirm these ...

journal_title：Prion

authors： Calero O,Bullido MJ,Clarimón J,Hortigüela R,Frank-García A,Martínez-Martín P,Lleó A,Rey MJ,Sastre I,Rábano A,de Pedro-Cuesta J,Ferrer I,Calero M

更新日期：2012-09-01 00:00:00

abstract：:We recently developed a new in vitro amplification technology, designated "real-time quaking-induced conversion (RT-QUIC)", for detection of the abnormal form of prion protein (PrPSc) in easily accessible specimens such as cerebrospinal fluid (CSF). After assessment of more than 200 CSF specimens from Japanese and Aus...

journal_title：Prion

authors： Atarashi R,Sano K,Satoh K,Nishida N

更新日期：2011-07-01 00:00:00

abstract：:Recent studies on iatrogenic Creutzfeldt-Jakob disease (CJD) raised concerns that one of the hallmark lesions of Alzheimer disease (AD), amyloid-β (Aβ), may be transmitted from human-to-human. The neuropathology of AD-related lesions is complex. Therefore, many aspects need to be considered in deciding on this issue. ...

journal_title：Prion

authors： Kovacs GG

更新日期：2016-09-02 00:00:00

abstract：:The detection method based on the mathematical expectation (ME) strategy is fast and accuracy for low frequency mutation screening in large samples. Previous studies have found that the 14-bp insertion/deletion (indel) variants of the 3' untranslated region (3' UTR) within bovine PRNP gene have been characterized with...

journal_title：Prion

authors： Yang Q,Zhang S,Liu L,Cao X,Lei C,Qi X,Lin F,Qu W,Qi X,Liu J,Wang R,Chen H,Lan X

更新日期：2016-09-02 00:00:00

abstract：:The objectives of this study were to test whether recombinant mouse (mo)PrP alone or in combination with LPS or under simulated endotoxemia would affect expression of genes related to host inflammatory and antimicrobial responses. To test our hypotheses colon tissues were collected from 16 male mice (FVB/N strain) and...

journal_title：Prion

authors： Dervishi E,Lam TH,Dunn SM,Zwierzchowski G,Saleem F,Wishart DS,Ametaj BN

更新日期：2015-01-01 00:00:00

abstract：:Prion and Alzheimer diseases are fatal neurodegenerative diseases caused by misfolding and aggregation of the cellular prion protein (PrP(C)) and the β-amyloid peptide, respectively. Soluble oligomeric species rather than large aggregates are now believed to be neurotoxic. PrP(C) undergoes three proteolytic cleavages ...

journal_title：Prion

authors： Béland M,Roucou X

更新日期：2014-01-01 00:00:00

abstract：:Each known abnormal prion protein (PrP (Sc) ) is considered to have a specific range and therefore the ability to infect some species and not others. Consequently, some species have been assumed to be prion disease resistant as no successful natural or experimental challenge infections have been reported. This assumpt...

journal_title：Prion

authors： Fernández-Borges N,Chianini F,Eraña H,Vidal E,Eaton SL,Pintado B,Finlayson J,Dagleish MP,Castilla J

更新日期：2012-11-01 00:00:00

abstract：:Synaptic dysfunction is a key process in the evolution of many neurodegenerative diseases, with synaptic loss preceding that of neuronal cell bodies. In Alzheimer, Huntington, and prion diseases early synaptic changes correlate with cognitive and motor decline, and altered synaptic function may also underlie deficits ...

journal_title：Prion

authors： Mallucci GR

更新日期：2009-10-01 00:00:00

abstract：:Yeast have been extensively used to model aspects of protein folding diseases, yielding novel mechanistic insights and identifying promising candidate therapeutic targets. In particular, the neurodegenerative disorder Huntington disease (HD), which is caused by the abnormal expansion of a polyglutamine tract in the hu...

journal_title：Prion

authors： Mason RP,Giorgini F

更新日期：2011-10-01 00:00:00

abstract：:Prion diseases are infectious conformational diseases. Despite the determination of many native prion protein (PrP) structures and in vitro production of infectious prions from recombinant PrP the structural background of PrP conversion remains the largest unsolved problem. The aggregated state of PrP (Sc) makes it in...

journal_title：Prion

authors： Hafner-Bratkovič I,Jerala R

更新日期：2011-04-01 00:00:00

abstract：:A case of L-type-like atypical bovine spongiform encephalopathy was detected in 14-year-old Japanese black beef cattle (BSE/JP24). To clarify the biological and biochemical properties of the prion in BSE/JP24, we performed a transmission study with wild-type mice and bovinized transgenic mice (TgBoPrP). The BSE/JP24 p...

journal_title：Prion

authors： Masujin K,Shu Y,Yamakawa Y,Hagiwara K,Sata T,Matsuura Y,Iwamaru Y,Imamura M,Okada H,Mohri S,Yokoyama T

更新日期：2008-07-01 00:00:00

abstract：:Bovine spongiform encephalopathy (BSE) created a global European crisis in the 1980s and 90s, with very serious health and economic implications. Classical BSE now appears to be under control, to a great extent as a result of a global research effort that identified the sources of prions in meat and bone meal (MBM) an...

journal_title：Prion

authors： Requena JR,Kristensson K,Korth C,Zurzolo C,Simmons M,Aguilar-Calvo P,Aguzzi A,Andreoletti O,Benestad SL,Böhm R,Brown K,Calgua B,Del Río JA,Espinosa JC,Girones R,Godsave S,Hoelzle LE,Knittler MR,Kuhn F,Legname G,La

更新日期：2016-05-03 00:00:00

abstract：:Studies of the ovine prion-related protein (testis-specific) gene (PRNT), including studies of genetic diversity, have highlighted its potential relationship to scrapie infection and economically important ovine traits. PRNT was previously reported to be highly polymorphic in Portuguese sheep. To characterize genetic ...

journal_title：Prion

authors： Li J,Zhang S,Erdenee S,Sun X,Dang R,Huang Y,Lei C,Chen H,Xu H,Cai Y,Lan X

更新日期：2018-01-01 00:00:00

abstract：:Prions, the causative agent of chronic wasting disease (CWD) enter the environment through shedding of bodily fluids and carcass decay, posing a disease risk as a result of their environmental persistence. Plants have the ability to take up large organic particles, including whole proteins, and microbes. This study us...

journal_title：Prion

authors： Rasmussen J,Gilroyed BH,Reuter T,Dudas S,Neumann NF,Balachandran A,Kav NN,Graham C,Czub S,McAllister TA

更新日期：2014-01-01 00:00:00

abstract：:The description of prions as causal agents of Transmissible Spongiform Encephalopathies (TSE), is nowadays accepted as an important breakthrough in biology as revealed the existence of a completely new group of pathogens and a new way of transmission for biological information. A common feature of many neurodegenerati...

journal_title：Prion

authors： Eraña H

更新日期：2019-01-01 00:00:00

abstract：:M2B cells with persistent classical bovine spongiform encephalopathy (C-BSE) have been established previously. In this study, we performed strain characterization of the M2B cell line in bovine PrPC overexpressing mice (Tg 1896). Mice intracranially inoculated with M2B cells and C-BSE survived for 451 ± 7 and 465 ± 31...

journal_title：Prion

authors： Suh TY,Roh IS,Kim HJ,Griffiths PC,Park KJ,Park HC,Hope J,Kang HE,Kim DY,Sohn HJ

更新日期：2017-11-02 00:00:00

abstract：OBJECTIVE:To evaluate whether EPC-MVs could promote bone regeneration by directly regulating osteoblast through miR-126. The underlying mechanisms were also explored. METHODS:EPCs were isolated from bone marrow mononuclear cells. EPC-MVs were collected from EPCs cultured medium. The lentivirus was used to induce miR-1...

journal_title：Prion

authors： Chen G,Li P,Liu Z,Zeng R,Ma X,Chen Y,Xu H,Li Z,Lin H

更新日期：2019-01-01 00:00:00

abstract：:The conformational diseases, linked to protein aggregation into amyloid conformations, range from non-infectious neurodegenerative disorders, such as Alzheimer disease (AD), to highly infectious ones, such as human transmissible spongiform encephalopathies (TSEs). They are commonly known as prion diseases. However, si...

journal_title：Prion

authors： Sabate R

更新日期：2014-01-01 00:00:00