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Chiara Vendramin, Siobhan McGuckin, Ferras Alwan, John-Paul Westwood, Mari Thomas, Marie Scully
BACKGROUND: Patients presenting with acute episodes of thrombotic microangiopathies (TMAs) require urgent access to plasma exchange (PEX). OctaplasLG, a solvent/detergent fresh-frozen plasma product that has undergone viral inactivation and prion reduction step, has been used in our institution since 2013, replacing Octaplas. STUDY DESIGN AND METHODS: We prospectively reviewed 981 PEX procedures where OctaplasLG was the replacement fluid in 90 patients admitted acutely with a TMA presentation within our institution from January 1, 2013, to December 31, 2015...
October 23, 2016: Transfusion
Seung-Hee Lee, Jaehoon Shim, Ye-Hwang Cheong, Sun-Lim Choi, Yong-Woo Jun, Sue-Hyun Lee, Yeon-Su Chae, Jin-Hee Han, Yong-Seok Lee, Jin-A Lee, Chae-Seok Lim, Kausik Si, Stefan Kassabov, Igor Antonov, Eric R Kandel, Bong-Kiun Kaang, Deok-Jin Jang
Two pharmacologically distinct types of local protein synthesis are required for synapse- specific long-term synaptic facilitation (LTF) in Aplysia: one for initiation and the other for maintenance. ApCPEB, a rapamycin sensitive prion-like molecule regulates a form of local protein synthesis that is specifically required for the maintenance of the LTF. However, the molecular component of the local protein synthesis that is required for the initiation of LTF and that is sensitive to emetine is not known. Here, we identify a homolog of ApCPEB responsible for the initiation of LTF...
October 22, 2016: Molecular Brain
Guillaume Van der Rest, Human Rezaei, Frédéric Halgand
Prion protein is involved in deadly neurodegenerative diseases. Its pathogenicity is linked to its structural conversion (α-helix to β-strand transition). However, recent studies suggest that prion protein can follow a plurality of conversion pathways, which hints towards different conformers that might coexist in solution. To gain insights on the plasticity of the ovine prion protein (PrP) monomer, wild type (A136, R154, Q171), mutants and deletions of ARQ were studied by traveling wave ion mobility experiments coupled to mass spectrometry...
October 18, 2016: Journal of the American Society for Mass Spectrometry
Francesco M Galassi, Maciej Henneberg, Frank J Rühli
No abstract text is available yet for this article.
November 2016: Lancet Neurology
Heidrun Maja Ries, Carmen Nussbaum-Krammer
A particular subgroup of protein-misfolding diseases, comprising Alzheimer's and Parkinson's disease, involves amyloidogenic proteins that can form alternative pathogenic conformations with a high tendency to self-assemble into oligomeric and fibrillar species. Although misfolded proteins have been clearly linked to disease, the exact nature of the toxic species remains highly controversial. Increasing evidence suggests that there is little correlation between the occurrence of macroscopic protein deposits and toxic phenotypes in affected cells and tissues...
October 15, 2016: Essays in Biochemistry
Yoshihiro Kino, Chika Washizu, Masaru Kurosawa, Mizuki Yamada, Hiroshi Doi, Toru Takumi, Hiroaki Adachi, Masahisa Katsuno, Gen Sobue, Geoffrey G Hicks, Nobutaka Hattori, Tomomi Shimogori, Nobuyuki Nukina
FUS/TLS is an RNA/DNA-binding protein associated with neurodegenerative diseases including amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Previously, we found that a prion-like domain in the N-terminus of FUS/TLS mediates co-aggregation between FUS/TLS and mutant huntingtin, the gene product of Huntington's disease (HD). Here, we show that heterozygous knockout of FUS/TLS worsened the phenotypes of model mice of (HD, but not spinal and bulbar muscular atrophy (SBMA). This difference was correlated with the degree of pathological association between disease proteins and FUS/TLS...
October 14, 2016: Scientific Reports
Mamoru Haratake, Tohru Takiguchi, Naho Masuda, Sakura Yoshida, Takeshi Fuchigami, Morio Nakayama
Sup35 is a prion-like protein from yeast and shares the ability to transmit its aberrant fold and to aggregate into amyloid fibrils. (7)GNNQQNY(13) from the prion-determining domain of Sup35 was reported to form an amyloid. We first investigated the self-aggregation transition behavior of GNNQQNY to the β-sheet amyloid state under various conditions. Mechanical stirring using a magnetic bar resulted in accelerated aggregation of the GNNQQNY. The aggregation rate of GNNQQNY was also dependent on its concentration; the higher the GNNQQNY concentration, the faster the aggregation...
October 7, 2016: Colloids and Surfaces. B, Biointerfaces
Fernando Bergasa-Caceres, Herschel A Rabitz
A simple analytical model is applied to study the effects of macromolecular crowding on the stability of partially folded states of the murine prion protein. It is found that relatively low levels of macromolecular crowding stabilize the partially folded states. The magnitude of the stabilization effect is similar for the partially folded to that of the fully folded state. Thus, the model suggests that it is on-pathway molten globule-like states, rather than partially folded states arising from unfolding of the native state, which play a key role in the pathogenic interconversion mechanism under crowded conditions...
October 13, 2016: Journal of Physical Chemistry. B
Matthias Schmitz, Maria Cramm, Franc Llorens, Dominik Müller-Cramm, Steven Collins, Ryuichiro Atarashi, Katsuya Satoh, Christina D Orrù, Bradley R Groveman, Saima Zafar, Walter J Schulz-Schaeffer, Byron Caughey, Inga Zerr
The development and adaption of in vitro misfolded protein amplification systems has been a major innovation in the detection of abnormally folded prion protein scrapie (PrP(Sc)) in human brain and cerebrospinal fluid (CSF) samples. Herein, we describe a fast and efficient protein amplification technique, real-time quaking-induced conversion (RT-QuIC), for the detection of a PrP(Sc) seed in human brain and CSF. In contrast to other in vitro misfolded protein amplification assays-such as protein misfolding cyclic amplification (PMCA)-which are based on sonication, the RT-QuIC technique is based on prion seed-induced misfolding and aggregation of recombinant prion protein substrate, accelerated by alternating cycles of shaking and rest in fluorescence plate readers...
November 2016: Nature Protocols
Andrew G Matveenko, Polina B Drozdova, Mikhail V Belousov, Svetlana E Moskalenko, Stanislav A Bondarev, Yury A Barbitoff, Anton A Nizhnikov, Galina A Zhouravleva
[PSI(+) ] is the prion form of the translation termination factor Sup35 (eRF3); [PSI(+) ] strains display nonsense suppression. Another prion-like element, [ISP(+) ], is linked to antisuppression in a specific background. Transcriptional regulator Sfp1 was shown to be responsible for [ISP(+) ] propagation. In this work, we identified SFP1 as a multicopy inducer of [PSI(+) ]-dependent lethality. Sfp1 is likely to up-regulate transcription of genes encoding release factors; however, its overproduction increases Sup35, but not Sup45 protein level...
October 12, 2016: Genes to Cells: Devoted to Molecular & Cellular Mechanisms
Alana M Thackray, Olivier Andreoletti, Raymond Bujdoso
In pursuit of a tractable bioassay to assess blood prion infectivity we have generated PrP transgenic Drosophila , which show a neurotoxic phenotype in adulthood after exposure to exogenous prions at the larval stage. Here we determined the sensitivity of ovine PrP transgenic Drosophila to ovine prion infectivity by exposure of these flies to a dilution series of scrapie-infected sheep brain homogenate. Ovine PrP transgenic Drosophila showed a significant neurotoxic response to dilutions of 10-2 through to 10-10 of the original scrapie-infected sheep brain homogenate...
October 12, 2016: Biochemical Journal
Lisa Gasperini, Elisa Meneghetti, Giuseppe Legname, Federico Benetti
Essential elements as copper and iron modulate a wide range of physiological functions. Their metabolism is strictly regulated by cellular pathways, since dysregulation of metal homeostasis is responsible for many detrimental effects. Neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease and prion diseases are characterized by alterations of metal ions. These neurodegenerative maladies involve proteins that bind metals and mediate their metabolism through not well-defined mechanisms. Prion protein, for instance, interacts with divalent cations via multiple metal-binding sites and it modulates several metal-dependent physiological functions, such as S-nitrosylation of NMDA receptors...
2016: Frontiers in Neuroscience
Indulekha P Sudhakaran, Mani Ramaswami
Long-term and short-term memories differ primarily in the duration of their retention. At a molecular level, long-term memory (LTM) is distinguished from short-term memory (STM) by its requirement for new gene expression. In addition to transcription (nuclear gene expression) the translation of stored mRNAs is necessary for LTM formation. The mechanisms and functions for temporal and spatial regulation of mRNAs required for LTM is a major contemporary problem, of interest from molecular, cell biological, neurobiological and clinical perspectives...
October 11, 2016: RNA Biology
Agata Mata, Laura Urrea, Silvia Vilches, Franc Llorens, Katrin Thüne, Juan-Carlos Espinosa, Olivier Andréoletti, Alejandro M Sevillano, Juan María Torres, Jesús Rodríguez Requena, Inga Zerr, Isidro Ferrer, Rosalina Gavín, José Antonio Del Río
Reelin is an extracellular glycoprotein involved in key cellular processes in developing and adult nervous system, including regulation of neuronal migration, synapse formation, and plasticity. Most of these roles are mediated by the intracellular phosphorylation of disabled-1 (Dab1), an intracellular adaptor molecule, in turn mediated by binding Reelin to its receptors. Altered expression and glycosylation patterns of Reelin in cerebrospinal and cortical extracts have been reported in Alzheimer's disease...
October 10, 2016: Molecular Neurobiology
Jamie C Fong, Julio C Rojas, Jee Bang, Andrea Legati, Katherine P Rankin, Sven Forner, Zachary A Miller, Anna M Karydas, Giovanni Coppola, Carrie K Grouse, Jeffrey Ralph, Bruce L Miller, Michael D Geschwind
Patients with pathogenic truncating mutations in the prion gene (PRNP) usually present with prolonged disease courses with severe neurofibrillary tangle and cerebral amyloidosis pathology, but more atypical phenotypes also occur, including those with dysautonomia and peripheral neuropathy. We describe the neurological, cognitive, neuroimaging, and electrophysiological features of a 31-year-old man presenting with an orbitofrontal syndrome, gastrointestinal symptoms, and peripheral neuropathy associated with PRNP Q160X nonsense mutation, with symptom onset at age 27...
October 4, 2016: Journal of Alzheimer's Disease: JAD
Mick F Tuite
A comprehensive analysis uncovered a set of yeast proteins promoting protein-based inheritance that shares many of the non-Mendelian properties of prions. Lacking any sequence or structural signatures of known prions, these proteins represent a new class of non-amyloid, protein-based epigenetic determinants that can control phenotype without impacting genotype.
October 6, 2016: Cell
Susanne Niedermeyer, Martin Eiden, Pavlos Toumazos, Penelope Papasavva-Stylianou, Ioannis Ioannou, Theodoros Sklaviadis, Cynthia Panagiotidis, Jan Langeveld, Alex Bossers, Thorsten Kuczius, Martin Kaatz, Martin H Groschup, Christine Fast
Scrapie and bovine spongiform encephalopathy (BSE) are transmissible spongiform encephalopathies (TSE's) affecting sheep and goats. Susceptibility of goats to scrapie is influenced by polymorphisms of the prion protein gene (PRNP) of the host. Five polymorphisms are associated with reduced susceptibility to TSE's. In the study presented here caprine samples from a scrapie eradication program on Cyprus were genotyped and further characterized using BioRad TeSeE rapid test, histological, immunohistochemical and biochemical methods...
October 6, 2016: Veterinary Research
Saida Abounit, Jessica W Wu, Guiliana Soraya Victoria, Chiara Zurzolo
The mechanisms of intercellular spreading of amyloidogenic proteins involved in neurodegenerative diseases have yet to be fully elucidated. While secretion has been implicated in the transfer of many proteins, including prions and α-synuclein, tunneling nanotubes (TNTs) have also been demonstrated for prions and mutant Huntingtin. Here, we provide further evidence that Tau aggregates, which have been demonstrated to predominantly be transferred via secretion, can also be found in TNTs. Additionally cells that have taken up Tau have increased TNT formation...
October 7, 2016: Prion
Stefanie Hackl, Alanca Schmid, Christian F W Becker
The site-selective installation of lipid modifications on proteins is critically important in our understanding of how membrane association influences the biophysical properties of proteins as well as to study certain proteins in their native environment. Here, we describe the use of split inteins for the C-terminal attachment of lipid-modified peptides to virtually any protein of interest (POI) via protein trans-splicing (PTS). To achieve this, the protein of interest is expressed in fusion with the N-terminal split intein segment and the C-terminal split intein segment is prepared by solid phase peptide synthesis...
2017: Methods in Molecular Biology
Rukmini Mukherjee, Oishee Chakrabarti
Health and homoeostasis are maintained by a dynamic balance between mitochondrial fission and fusion. Mitochondrial fusion machinery is largely unknown in mammals. Only a few reports have illustrated the role of Fzo1 in mitochondrial fusion known in Saccharomyces cerevisiae. We demonstrate that the ubiquitin ligase Mahogunin Ring Finger-1 (MGRN1) interacts with and constitutively ubiquitinates the mammalian homolog, Mitofusin1 (Mfn1) via K63 linkages. In mice models, loss of Mgrn1 function leads to severe developmental defects and adult-onset spongiform neurodegeneration, similar to prion diseases...
October 4, 2016: Biochimica et Biophysica Acta
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