Results 41 to 50 of about 92,941 (293)

RETRACTED ARTICLE: Neurotropic influenza A virus infection causes prion protein misfolding into infectious prions in neuroblastoma cells

Scientific Reports, 2021
Misfolding of the cellular prion protein, PrPC, into the amyloidogenic isoform, PrPSc, which forms infectious protein aggregates, the so-called prions, is a key pathogenic event in prion diseases.
Hideyuki Hara, Junji Chida, Keiji Uchiyama, Agriani Dini Pasiana, Etsuhisa Takahashi, Hiroshi Kido, Suehiro Sakaguchi   +6 more
doaj   +1 more source

Prion protein misfolding and disease [PDF]

Current Opinion in Structural Biology, 2009
Transmissible spongiform encephalopathies (TSEs or prion diseases) are a rare group of invariably fatal neurodegenerative disorders that affect humans and other mammals. TSEs are protein misfolding diseases that involve the accumulation of an abnormally aggregated form of the normal host prion protein (PrP).
Roger A, Moore, Lara M, Taubner, Suzette A, Priola   +2 more
openaire   +2 more sources

Mitochondrial stress in advanced fibrosis and cirrhosis associated with chronic hepatitis B, chronic hepatitis C, or nonalcoholic steatohepatitis

Hepatology, EarlyView., 2022
Adaptive mitochondrial mechanisms allow mitochondrial resilience and prevent the worsening of fibrosis, while deregulation of these mechanisms promotes the progression from no/minimal‐mild (F0‐F2) fibrosis to advanced fibrosis and cirrhosis (F3‐F4). Abstract Background and Aims Hepatitis B virus (HBV) infection causes oxidative stress (OS) and alters ...
Dimitri Loureiro, Issam Tout, Stéphanie Narguet, Cheikh Mohamed Bed, Morgane Roinard, Ahmad Sleiman, Nathalie Boyer, Nathalie Pons‐Kerjean, Corinne Castelnau, Nathalie Giuly, Dorothy Tonui, Vassili Soumelis, Jamel El Benna, Patrick Soussan, Richard Moreau, Valérie Paradis, Abdellah Mansouri, Tarik Asselah   +17 more
wiley   +1 more source

All-atom simulations reveal how single point mutations promote serpin misfolding

, 2018
Protein misfolding is implicated in many diseases, including the serpinopathies. For the canonical inhibitory serpin {\alpha}1-antitrypsin (A1AT), mutations can result in protein deficiencies leading to lung disease, and misfolded mutants can accumulate ...
Beccara, Silvio a, Faccioli, Pietro, Gershenson, Anne, Ianeselli, Alan, Orioli, Simone, Spagnolli, Giovanni, Wang, Fang, Wintrode, Patrick L.   +7 more
core   +1 more source

Folding of small disulfide-rich proteins : clarifying the puzzle [PDF]

, 2006
Premi a l'excel·lència investigadora. Àmbit de les Ciències Experimentals. 2008The process by which small proteins fold to their native conformations has been intensively studied over the last few decades.
Apuy, Arolas, Arolas, Arolas, Arolas, Baneyx, Bardwell, Cemazar, Cemazar, Chang, Chang, Chang, Chang, Chang, Chang, Chang, Chang, Chang, Chang, Chang, Chatrenet, Creighton, Creighton, Creighton, Creighton, Creighton, Daggett, Daly, Darby, Dobson, Ellgaard, Francesc X. Aviles, Fuller, Gehrmann, Goldberg, Goldenberg, Heitz, Hogg, Houry, Joan L. Arolas, Jui-Yoa Chang, Klein-Seetharaman, Kortemme, Kuhlman, Laity, Li, Lu, Matagne, Narayan, Narayan, Narayan, Narayan, Onuchic, Permyakov, Ross, Ruoppolo, Ruoppolo, Salamanca, Salvador Ventura, Scheraga, Shimotakahara, Shin, van den Berg, van den Berg, van Mierlo, van Mierlo, Venhudova, Wedemeyer, Weissman, Welker, Welker, Wu, Yang, Zavodszky   +73 more
core   +2 more sources

Molecular mechanisms of proteinopathies across neurodegenerative disease: a review

Neurological Research and Practice, 2019
Background Although there is a range of different symptoms across neurodegenerative diseases, they have been noted to have common pathogenic features.
Alexander P. Marsh
doaj   +1 more source

The common truncation variant in pancreatic lipase related protein 2 (PNLIPRP2) is expressed poorly and does not alter risk for chronic pancreatitis [PDF]

, 2018
A nonsense variant (p.W358X) of human pancreatic lipase related protein 2 (PNLIPRP2) is present in different ethnic populations with a high allele frequency.
Hegyi, Eszter, Hegyi, Peter, Lowe, Mark E, Nemeth, Balazs Csaba, Pesei, Zsofia Gabriella, Sahin-Toth, Miklos, Szentesi, Andrea, Szucs, Akos   +7 more
core   +2 more sources

Alpha-Synuclein Oligomers Interact with Metal Ions to Induce Oxidative Stress and Neuronal Death in Parkinson's Disease [PDF]

, 2015
Protein aggregation and oxidative stress are both key pathogenic processes in Parkinson's disease, although the mechanism by which misfolded proteins induce oxidative stress and neuronal death remains unknown.
Andrey Y. Abramov, Blerida Banushi, Christopher M. Dobson, Daniel Little, David Klenerman, Dias V, Emma Deas, Marthe H.R. Ludtmann, Mathew H. Horrocks, Michael J. Devine, Nicholas W. Wood, Nunilo Cremades, Paul Gissen, Plamena R. Angelova, Serene Chen, Sonia Gandhi, Zhi Yao   +16 more
core   +5 more sources

Abnormal degradation of the neuronal stress-protective transcription factor HSF1 in Huntington’s disease

Nature Communications, 2017
Huntington’s disease (HD) is caused by misfolding of mutant Htt protein. The authors find that in HD models, the decreased expression of heat shock transcription factor 1 that usually protects against protein misfolding, is in part caused by elevated ...
Rocio Gomez-Pastor, Eileen T. Burchfiel, Daniel W. Neef, Alex M. Jaeger, Elisa Cabiscol, Spencer U. McKinstry, Argenia Doss, Alejandro Aballay, Donald C. Lo, Sergey S. Akimov, Christopher A. Ross, Cagla Eroglu, Dennis J. Thiele   +12 more
doaj   +1 more source

Heat shock-induced phosphorylation of TAR DNA-binding protein 43 (TDP-43) by MAPK/ERK kinase regulates TDP-43 function [PDF]

, 2017
TAR DNA-binding protein (TDP-43) is a highly conserved and essential DNA- and RNA-binding protein that controls gene expression through RNA processing, in particular, regulation of splicing.
Ayala, Yuna M, Bigio, Eileen H, Fey, Erin E, French, Rachel L, Knoverek, Catherine R, Li, Wen, Lin, Binyan, Reeb, Ashley N, Subramanian, Praveen   +8 more
core   +2 more sources