STXBP1 forms amyloid-like aggregates in rat brain and demonstrates amyloid properties in bacterial expression system.

abstract：:Insulin-degrading enzyme (IDE) is a conserved Zn(2+)metalloendopeptidase involved in insulin degradation and in the maintenance of brain steady-state levels of amyloid beta peptide (Abeta) of Alzheimer's disease (AD). Our recent demonstration that IDE and Abeta are capable of forming a stoichiometric and extremely sta...

journal_title：Prion

authors： de Tullio MB,Morelli L,Castaño EM

更新日期：2008-04-01 00:00:00

abstract：:Amyloid refers to the abnormal fibrous, extracellular, proteinaceous deposits found in organs and tissues. Amyloid is insoluble and is structurally dominated by beta-sheet structure. Unlike other fibrous proteins it does not commonly have a structural, supportive or motility role but is associated with the pathology s...

journal_title：Prion

authors： Rambaran RN,Serpell LC

更新日期：2008-07-01 00:00:00

abstract：:Nucleic acid sequences of the prion gene (PRNP) were examined and genotypes compiled for 76 white-tailed deer from northern Illinois, which previously tested positive for chronic wasting disease (CWD), and 120 negative animals selected to control for geographic location and age. Nine nucleotide polymorphisms, seven si...

journal_title：Prion

authors： Kelly AC,Mateus-Pinilla NE,Diffendorfer J,Jewell E,Ruiz MO,Killefer J,Shelton P,Beissel T,Novakofski J

更新日期：2008-01-01 00:00:00

abstract：:Exposure to high temperature or other stresses induces a synthesis of heat shock proteins. Many of these proteins are molecular chaperones, and some of them help cells to cope with heat-induced denaturation and aggregation of other proteins. In the last decade, chaperones have received increased attention in connectio...

journal_title：Prion

authors： Rikhvanov EG,Romanova NV,Chernoff YO

更新日期：2007-10-01 00:00:00

abstract：:Although intensively researched, the fundamental mechanism of protein misfolding that leads to protein aggregation and associated diseases remains somewhat enigmatic. The failure of a protein to correctly fold de novo or to remain correctly folded can have profound consequences on a living system especially when the c...

journal_title：Prion

authors： Tuite MF,Melki R

更新日期：2007-04-01 00:00:00

abstract：:Proteins are a major constituent of cells with specific biological functions. Besides the primary structure that is simply the sequence of amino acids that comprise a protein, the secondary structure represents the first step of folding defining its general conformation. The biological functions of proteins are direct...

journal_title：Prion

authors： Lupi O,Peryassu MA

更新日期：2007-10-01 00:00:00

abstract：:Infectious amyloid forms of the release factor, Sup35, comprise the yeast prion [PSI+]. This protein-based unit of inheritance is an evolutionary capacitor able to release cryptic genetic variation during environmental stress and generate potentially beneficial phenotypes. Genetic data have uncovered a sophisticated p...

journal_title：Prion

authors： Sweeny EA,Shorter J

更新日期：2008-10-01 00:00:00

abstract：:Despite intensive research into how amyloid structures can impair cellular viability, the molecular nature of these toxic species and the cellular mechanisms involved are not clearly defined and may differ from one disease to another. We systematically analyzed, in Saccharomyces cerevisiae, genes that increase the tox...

journal_title：Prion

authors： Couthouis J,Marchal C,D'Angelo F,Berthelot K,Cullin C

更新日期：2010-10-01 00:00:00

abstract：:Genetic Creutzfeldt-Jakob disease (gCJD) is caused by a range of mutations in the prion protein gene (PRNP) and account for approximately 10-15% of overall human prion diseases worldwide. They are different with disease onset, disease duration, clinical signs and diagnostic findings. Here we reported a 71 year-old fem...

journal_title：Prion

authors： Shi Q,Chen C,Song XN,Gao C,Tian C,Zhou W,Song XH,Yao LS,Han J,Dong XP

更新日期：2011-04-01 00:00:00

abstract：:A case of Creutzfeldt-Jakob disease (CJD) with a rare mutation of the prion protein (PrP) gene (PRNP) at codon 208 (R208H), while the codon 129 was a methionine homozygous genotype is reported. The patient initial displayed hand tremor, dizziness and progressive cognitive dysfunction. Subsequently, other symptoms grad...

journal_title：Prion

authors： Chen C,Shi Q,Tian C,Li Q,Zhou W,Gao C,Han J,Dong XP

更新日期：2011-07-01 00:00:00

abstract：:The subacute spongiform encephalopathies are prion diseases characterized by acute and rapid neurodegeneration that lead to the death of the patient within months to a few years. The epidemiology of CJD is complicated and the frequency in Mexico is unknown. We aim to describe the cases of prion disease in Mexico. Cons...

journal_title：Prion

authors： González-Duarte A,Medina Z,Balaguer RR,Calleja JH

更新日期：2011-07-01 00:00:00

abstract：:Knowledge of the natural roles of cellular prion protein (PrP (C) ) is essential to an understanding of the molecular basis of prion pathologies. This GPI-anchored protein has been described in synaptic contacts, and loss of its synaptic function in complex systems may contribute to the synaptic loss and neuronal dege...

journal_title：Prion

authors： Llorens F,Del Río JA

更新日期：2012-07-01 00:00:00

abstract：:Prion diseases comprise a family of fatal neurodegenerative disorders caused by the conformational re-arrangement of a normal host-encoded protein, PrP (C) , to an abnormal infectious isoform termed PrP (Sc) . Currently, the precise cellular mechanism(s) underlying prion disease pathogenesis remain unclear. Evidence s...

journal_title：Prion

authors： Andre R,Tabrizi SJ

更新日期：2012-01-01 00:00:00

abstract：:Chronic wasting disease (CWD) is an invariably fatal neurologic disease that naturally infects mule deer, white tailed deer and elk. The understanding of CWD neurodegeneration at a molecular level is very limited. In this study, microarray analysis was performed to determine changes in the gene expression profiles in ...

journal_title：Prion

authors： Basu U,Almeida LM,Dudas S,Graham CE,Czub S,Moore SS,Guan LL

更新日期：2012-07-01 00:00:00

abstract：:Soluble oligomeric amyloid-β (Aβ) has been suggested to impair synaptic and neuronal function, leading to neurodegeneration that is clinically observed as the memory and cognitive dysfunction characteristic of Alzheimer disease, while the precise mechanism(s) whereby oligomeric Aβ causes neurotoxicity remains unknown....

journal_title：Prion

authors： Kudo W,Petersen RB,Lee HG

更新日期：2013-03-01 00:00:00

abstract：:Over the past decade, immunohistochemical studies have provided compelling evidence that gray matter (GM) pathology in multiple sclerosis (MS) is extensive. Until recently, this GM pathology was difficult to visualize using standard magnetic resonance imaging (MRI) techniques. However, with newly developed MRI sequenc...

journal_title：Prion

authors： Klaver R,De Vries HE,Schenk GJ,Geurts JJ

更新日期：2013-01-01 00:00:00

abstract：:Prion diseases are associated with misfolding of the natively α-helical prion protein into isoforms that are rich in cross β-structure. However, both the mechanism by which pathological conformations are produced and their structural properties remain unclear. Using a combination of nuclear magnetic resonance spectros...

journal_title：Prion

authors： Zweckstetter M

更新日期：2013-05-01 00:00:00

abstract：:A significant body of evidence shows that polyglutamine (polyQ) tracts are important for various biological functions. The characteristic polymorphism of polyQ length is thought to play an important role in the adaptation of organisms to their environment. However, proteins with expanded polyQ are prone to form amyloi...

journal_title：Prion

authors： Alexandrov AI,Ter-Avanesyan MD

更新日期：2013-05-01 00:00:00

abstract：:The prion responsible for the Bovine Spongiform Encephalopathy (BSE) shows unique features when compared with other prions. One of these features is its ability to infect almost all experimentally tested animal models. In the paper published in The Journal of Neuroscience (1) we describe a series of experiments direct...

journal_title：Prion

authors： Vidal E,Fernández-Borges N,Pintado B,Ordóñez M,Márquez M,Fondevila D,Eraña H,Torres JM,Pumarola M,Castilla J

更新日期：2013-11-01 00:00:00

abstract：:Neurodegenerative diseases belong to a larger group of protein misfolding disorders, known as proteinopathies. There is increasing experimental evidence implicating prion-like mechanisms in many common neurodegenerative disorders, including Alzheimer disease, Parkinson disease, the tauopathies, and amyotrophic lateral...

journal_title：Prion

authors： Grad LI,Cashman NR

更新日期：2014-01-01 00:00:00

abstract：:A direct physical interaction of the prion protein isoforms is a key element in prion conversion. Which sites interact first and which parts of PrP(c) are converted subsequently is presently not known in detail. We hypothesized that structural changes induced by PrP(Sc) interaction occur in more than one interface and...

journal_title：Prion

authors： Taguchi Y,Schätzl HM

更新日期：2013-11-01 00:00:00

abstract：:A baffling aspect of metazoan proteostasis is the lack of an Hsp104 ortholog that rapidly disaggregates and reactivates misfolded polypeptides trapped in stress induced disordered aggregates, preamyloid oligomers, or amyloid fibrils. By contrast, in bacteria, protozoa, chromista, fungi, and plants, Hsp104 orthologs ar...

journal_title：Prion

authors： Torrente MP,Shorter J

更新日期：2013-11-01 00:00:00

abstract：:Prions are molecular pathogens, able to convert a normal cellular prion protein (PrP(C)) into a prion (PrP(Sc)). The information necessary for this conversion is contained in the conformation of PrP(Sc). Mass spectrometry (MS) and small-molecule covalent reactions have been used to study prions. Mass spectrometry has ...

journal_title：Prion

authors： Silva CJ

更新日期：2014-01-01 00:00:00

abstract：:In several recent studies transmissible prion disease was induced in animals by inoculation with recombinant prion protein amyloid fibrils produced in vitro. Serial transmission of amyloid fibrils gave rise to a new class of prion strains of synthetic origin. Gradual transformation of disease phenotypes and PrP(Sc) pr...

journal_title：Prion

authors： Baskakov IV

更新日期：2014-03-01 00:00:00

abstract：:The structures of the infectious prion protein, PrP(Sc), and that of its proteolytically truncated variant, PrP 27-30, have evaded experimental determination due to their insolubility and propensity to aggregate. Molecular modeling has been used to fill this void and to predict their structures, but various modeling a...

journal_title：Prion

authors： Requena JR,Wille H

更新日期：2014-01-01 00:00:00

abstract：:A typical feature of scrapie in sheep and goats is the accumulation of disease-associated prion protein. Scrapie consists of many strains with different biological properties. Nine natural sheep scrapie cases were transmitted to wild-type mice and mouse-passaged isolates were classified into 2 types based on incubatio...

journal_title：Prion

authors： Miyazawa K,Okada H,Iwamaru Y,Masujin K,Yokoyama T

更新日期：2014-01-01 00:00:00

abstract：:We report a Japanese patient with Creutzfeldt-Jakob disease (CJD) with a V203I homozygous mutation of the prion protein gene (PRNP). A 73-year-old woman developed rapidly progressive gait disturbance and cognitive dysfunction. Four months after the onset, she entered a state of an akinetic mutism. Gene analysis reveal...

journal_title：Prion

authors： Komatsu J,Sakai K,Hamaguchi T,Sugiyama Y,Iwasa K,Yamada M

更新日期：2014-01-01 00:00:00

abstract：:Prion proteins (PrP(C)) are cell membrane glycoproteins that can be found in many cell types, but specially in neurons. Many studies have suggested PrP(C)'s participation in metal transport and cellular protection against stress in the central nervous system (CNS). On the other hand PrP(Sc), the misfolded isoform of P...

journal_title：Prion

authors： Rivera NA,Novakofski J,Weng HY,Kelly A,Satterthwaite-Phillips D,Ruiz MO,Mateus-Pinilla N

更新日期：2015-01-01 00:00:00

abstract：:Yeast [PSI(+)] prion is one of the most suitable and well characterized system for the investigation of the prion phenomenon. However, until recently, the lack of data on the 3D arrangement of Sup35p prion fibrils hindered progress in this area. The recent arrival in this field of new experimental techniques led to th...

journal_title：Prion

authors： Bondarev SA,Zhouravleva GA,Belousov MV,Kajava AV

更新日期：2015-01-01 00:00:00

abstract：:Prions are infectious proteins and over the past few decades, some prions have become renowned for their causative role in several neurodegenerative diseases in animals and humans. Since their discovery, the mechanisms and mode of transmission and molecular structure of prions have begun to be established. There is, h...

journal_title：Prion

authors： Atkinson CJ,Zhang K,Munn AL,Wiegmans A,Wei MQ

更新日期：2016-01-01 00:00:00