Aβ seeds and prions: How close the fit?

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：: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：: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

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：: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：:Mammalian prions are composed of misfolded aggregated prion protein (PrP) with amyloid-like features. Prions are zoonotic disease agents that infect a wide variety of mammalian species including humans. Mammals and by-products thereof which are frequently encountered in daily life are most important for human health. ...

journal_title：Prion

authors： Hammarström P,Nyström S

更新日期：2015-01-01 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：: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：:We report here on the proceedings of the Global Alzheimer Summit that took place September 22-23, 2011 in Madrid, Spain. As Alzheimer disease (AD) is the leading cause of neurodegeneration in elderly individuals and as yet has no effective therapeutic option, it continues to stimulate global research interests. At the...

journal_title：Prion

authors： Gonsalves D,Jovanovic K,Da Costa Dias B,Weiss SF

更新日期：2012-01-01 00:00:00

abstract：:The molecular chaperone network plays a critical role in the formation and propagation of self-replicating yeast prions. Not only do individual prions differ in their requirements for certain chaperones, but structural variants of the same prion can also display distinct dependences on the chaperone machinery, specifi...

journal_title：Prion

authors： Dulle JE,True HL

更新日期：2013-09-01 00:00:00

abstract：:Modified nucleosides in tRNA anticodon loops such as 5-methoxy-carbonyl-methyl-2-thiouridine (mcm5s2U) and pseuduridine (Ψ) are thought to be required for an efficient decoding process. In Saccharomyces cerevisiae, the simultaneous presence of mcm5s2U and Ψ38 in tRNAGlnUUG was shown to mediate efficient synthesis of t...

journal_title：Prion

authors： Schaffrath R,Klassen R

更新日期：2017-01-02 00:00:00

abstract：:Among different types of protein aggregation, amyloids are a biochemically well characterized state of protein aggregation that are associated with a large number of neurodegenerative diseases including Parkinson's disease, Alzheimer and Creutzfeldt-Jakob disease. Yeast, Saccharomyces cerevisiae is an insightful model...

journal_title：Prion

authors： Aslam K,Hazbun TR

更新日期：2016-03-03 00:00:00

abstract：:The evolutionary origins of vertebrate prion genes had remained elusive until recently when multiple lines of evidence converged to the proposition that members of the prion gene family represent an ancient branch of a larger family of ZIP metal ion transporters. (1) A follow-up investigation which explored the mechan...

journal_title：Prion

authors： Ehsani S,Mehrabian M,Pocanschi CL,Schmitt-Ulms G

更新日期：2012-09-01 00:00:00

abstract：:The yeast, fungal and mammalian prions determine heritable and infectious traits that are encoded in alternative conformations of proteins. They cause lethal sporadic, familial and infectious neurodegenerative conditions in man, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), ...

journal_title：Prion

authors： Safar JG

更新日期：2012-04-01 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：: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：: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：: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：:Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are clinically overlapping neurodegenerative disorders whose pathophysiology remains incompletely understood. ALS initiates in a discrete location, and typically progresses in a pattern consistent with spread of the degenerative process t...

journal_title：Prion

authors： Udan M,Baloh RH

更新日期：2011-01-01 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：:It has been described that the breakdown of β-sheets in PrP (Sc) by denaturation results in loss of infectivity and PK-sensitivity, suggesting a relationship between the structure and PK-resistance. It is also known that an important fraction of total PrP (Sc) is PK-sensitive and can be isolated by the method we alr...

journal_title：Prion

authors： Sajnani G,Requena JR

更新日期：2012-11-01 00:00:00

abstract：:Many laboratory studies and epidemiological observations confirm that nematodes prevent some immune-mediated diseases. The development of immunologically well-defined laboratory models of intestinal nematode infection has allowed significant advances to be made in understanding the immunological basis of effector mech...

journal_title：Prion

authors： Donskow-Łysoniewska K,Brodaczewska K,Doligalska M

更新日期：2013-07-01 00:00:00

abstract：:In most human and animal prion diseases the abnormal disease-associated prion protein (PrPSc) is deposited as non-amyloid aggregates in CNS, spleen and lymphoid organs. In contrast, in humans and transgenic mice with PrP mutations which cause expression of PrP lacking a glycosylphosphatidylinositol (GPI)-anchor, most ...

journal_title：Prion

authors： Race B,Jeffrey M,McGovern G,Dorward D,Chesebro B

更新日期：2017-07-04 00:00:00

abstract：:Prion protein (PrP), the causative agent of transmissible spongiform encephalopathies, is synthesized in the endoplasmic reticulum (ER) where it undergoes numerous covalent modifications. Here we investigate the interdependence and regulation of PrP oxidative folding, N-glycosylation and GPI addition in diverse ER con...

journal_title：Prion

authors： Orsi A,Sitia R

更新日期：2007-10-01 00:00:00

abstract：:In Transmissible Spongiform Encephalopathies (TSEs) and Alzheimer disease (AD) both misfolding and aggregation of specific proteins represent key features. Recently, it was observed that PrP (c) is a mediator of a synaptic dysfunction induced by Aβ oligomers. We tested a novel γ secretase modulator (CHF5074) in a mur...

journal_title：Prion

authors： Poli G,Corda E,Lucchini B,Puricelli M,Martino PA,Dall'ara P,Villetti G,Bareggi SR,Corona C,Vallino Costassa E,Gazzuola P,Iulini B,Mazza M,Acutis P,Mantegazza P,Casalone C,Imbimbo BP

更新日期：2012-01-01 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：:Transmissible spongiform encephalopathies (TSEs) are a group of lethal neurodegenerative diseases involving the structural conversion of cellular prion protein (PrPC) into the pathogenic isoform (PrPSc) for which no effective treatment is currently available. Previous studies have implicated that a polymeric molecule ...

journal_title：Prion

authors： Honda R,Yamaguchi KI,Elhelaly AE,Fuji M,Kuwata K

更新日期：2018-01-01 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：: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：:TDP-43 and FUS are DNA/RNA binding proteins associated with neuronal inclusions in amyotrophic lateral sclerosis (ALS) patients. Other neurodegenerative diseases are also characterized by neuronal protein aggregates, e.g. Huntington's disease, associated with polyglutamine (polyQ) expansions in the protein huntingtin....

journal_title：Prion

authors： Park SK,Arslan F,Kanneganti V,Barmada SJ,Purushothaman P,Verma SC,Liebman SW

更新日期：2018-01-02 00:00:00