Results 101 to 110 of about 4,254,953 (206)

A novel protective prion protein variant that colocalizes with kuru exposure. [PDF]

, 2009
BACKGROUND: Kuru is a devastating epidemic prion disease that affected a highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly affected adult women and children of both sexes.
Whittaker, John, Mein, C., Shah, Paresh, Campbell, T., Whitfield, Jerome, Mein, Charles A, Whittaker, J., Alpers, Michael Philip, Hummerich, H., Collinge, J., Hummerich, Holger, Collinge, John, Uphill, J., Poulter, Mark, Beck, Jon, Al-Dujaily, Huda, Mead, S., Al-Dujaily, H., Alpers, Michael P, Beck, J., Verzilli, C., Mead, Simon, Verzilli, Claudio, Shah, P., Uphill, James, Poulter, M., Campbell, Tracy   +26 more
core   +1 more source

Development of a sensitive cell culture system to assess prion infectivity and the efficacy of prion decontamination technologies

, 2012
Creutzfeldt-Jakob disease (CJD) can be iatrogenically transmitted during transplants, grafts and transfusions from CJD infected donors and also contaminated surgical instruments.
Secker, Thomas
core   +1 more source

α‐Synuclein strain homogeneity in multiple system atrophy subtypes

Brain Pathology, EarlyView.
Two different subtypes of multiple system atrophy are recognized: MSA‐C and MSA‐P. In this manuscript, Lau et al. investigate whether MSA‐C and MSA‐P are caused by different strains of α‐synuclein aggregates. By performing conformational fingerprinting experiments as well as in vitro and in vivo seeding assays, their findings suggest that both MSA‐C ...
Heather H. C. Lau, Nicholas R. G. Silver, Surabhi Mehra, Raphaella W. L. So, Le yao Li, Alison Mao, Erica Stuart, Cian Schmitt‐Ulms, Bradley T. Hyman, Martin Ingelsson, Joel C. Watts   +10 more
wiley   +1 more source

Molecular dynamics simulations elucidate the misfolding mechanisms of secretion‐defective pancreatic lipase variants

The FEBS Journal, EarlyView.
Misfolding mutations in pancreatic lipase have been identified as potential contributors of chronic pancreatitis, an inflammatory disease of the human pancreas. Here, we describe the effect of these misfolding mutations on pancreatic lipase structure using molecular dynamics simulations and structural modeling.
Gyula Hoffka, András Szabó
wiley   +1 more source

Die Rolle von PrPC in der neuronalen Differenzierung und die Ausbreitung der infektiösen Isoform PrPSc durch Mikrovesikel [PDF]

, 2008
Prion diseases or transmissible spongiform encephalopathies (TSEs) are rare neurological disorders that may be of genetic or infectious origin, but most frequently occur sporadically in humans. Their outcome is invariably fatal.
Barenco Montrasio, Maria Grazia
core  

Gene expression profiling en association with prion-related lesions in the medulla oblongata of symptomatic natural scrapie animals. [PDF]

, 2011
The pathogenesis of natural scrapie and other prion diseases remains unclear. Examining transcriptome variations in infected versus control animals may highlight new genes potentially involved in some of the molecular mechanisms of prion-induced ...
Bossers, A., Rosa Bolea, Hicham Filali, Varona, L., Filalil, Hicham, Harders, F., Inmaculada Martin-Burriel, Alex Bossers, Zaragoza, P., Badiola, J.J., Juan J. Badiola, Bossers, Alex, Pumarola i Batlle, Martí, Lyahyai, Jaber, Lyahyai, J., Martín Burriel, Inmaculada, Pilar Zaragoza, Juan J Badiola, Bolea, Rosa, Filali, H., Bolea, R., Acín, Cristina, Luis Varona, Frank Harders, Filali, Hicham, Pumarola, M., Harders, Frank, Zaragoza, Pilar, Badiola, Juan J, Varona, Luis, Martí Pumarola, Jaber Lyahyai, Martin-Burriel, I., Badiola, Juan José, Pumarola, Martí   +34 more
core   +2 more sources

Constrictions and shear stress are key determinants of amyloidogenic light chain (AL) amyloidosis

The FEBS Journal, EarlyView.
In this study, we investigate how flow constrictions and shear stress trigger cardiac antibody light chain (AL) fibril formation and deposition. We mimic the mechanical forces exerted by the heart and the microvasculature network using a mini‐peristaltic pump setup and a grid‐type microchannel network, respectively.
Yuji Goto, Keiichi Yamaguchi, Kichitaro Nakajima, Ikuko Yumen, Ruiming He, Hirokazu Matsuda, Tetsuichi Wazawa, Takeharu Nagai, Nobuhiro Tsukada, Suguru Yamamoto, Johannes Buchner, Hirotsugu Ogi   +11 more
wiley   +1 more source

Analysis of the hippocampal proteome in ME7 prion disease reveals a predominant astrocytic signature and highlights the brain-restricted production of clusterin in chronic neurodegeneration

, 2014
Prion diseases are characterized by accumulation of misfolded protein, gliosis, synaptic dysfunction, and ultimately neuronal loss. This sequence, mirroring key features of Alzheimer disease, is modeled well in ME7 prion disease.
Asuni, Ayodeji A, Skipp, Paul, Gray, Bryony, Bailey, Joanne, O'Connor, Vincent, Perry, V Hugh   +5 more
core   +1 more source

CHAPTER 1.1. Disulfide Bonds in Protein Folding and Stability [PDF]

, 2018
Disulfide bonds are unique among post-translational modifications, as they add covalent crosslinks to the polypeptide chain. Accordingly, they can exert pronounced effects on protein folding and stability. This is of particular importance for secreted or
Sub Cellular Protein Chemistry, Hendershot, Linda M., Feige, Matthias J., Ineke Braakman, Matthias J. Feige, Braakman, Ineke, Cellular Protein Chemistry, Linda M. Hendershot   +7 more
core   +1 more source

Bone Grafts: Everything You Need to Know

Journal of Periodontal Research, EarlyView.
This review classifies bone grafts (autograft, allograft, xenograft, synthetic) by source and osteogenic/inductive/conductive potential, linking material properties and mechanisms to clinical indications in periodontal and dental regeneration. Key challenges—limited osteoinduction, poor vascularisation, unbalanced resorption, handling difficulties, and
Håvard Jostein Haugen, Javier Sanz, Giuseppe Perale, Augustine Mark Saiz, Mario Romandini, Maryam Rahmati   +5 more
wiley   +1 more source