New insights into structural determinants of prion protein folding and stability

Prion
Federico Benetti, Giuseppe Legname

Abstract

Prions are the etiological agent of fatal neurodegenerative diseases called prion diseases or transmissible spongiform encephalopathies. These maladies can be sporadic, genetic or infectious disorders. Prions are due to post-translational modifications of the cellular prion protein leading to the formation of a β-sheet enriched conformer with altered biochemical properties. The molecular events causing prion formation in sporadic prion diseases are still elusive. Recently, we published a research elucidating the contribution of major structural determinants and environmental factors in prion protein folding and stability. Our study highlighted the crucial role of octarepeats in stabilizing prion protein; the presence of a highly enthalpically stable intermediate state in prion-susceptible species; and the role of disulfide bridge in preserving native fold thus avoiding the misfolding to a β-sheet enriched isoform. Taking advantage from these findings, in this work we present new insights into structural determinants of prion protein folding and stability.

References

Feb 15, 1995·Biochemical and Biophysical Research Communications·M P HornshawJ M Candy
Dec 1, 1993·Proceedings of the National Academy of Sciences of the United States of America·K M PanF E Cohen
Nov 13, 1998·Proceedings of the National Academy of Sciences of the United States of America·S B Prusiner
Jul 5, 2001·Proceedings of the National Academy of Sciences of the United States of America·G S JacksonJ Collinge
Apr 13, 2002·Neurobiology of Disease·Marin GuentchevHerbert Budka
Mar 12, 2004·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Surachai Supattapone
May 25, 2004·Proceedings of the National Academy of Sciences of the United States of America·Cédric GovaertsFred E Cohen
Mar 22, 2005·Gene·Marko PremzlJennifer A Marshall Graves
Jun 30, 2006·Experimental Neurology·Seong-Wook YunMichael A Klein
May 9, 2009·PLoS Pathogens·Zrinka MarijanovicChiara Zurzolo
Nov 6, 2009·Prion·Federico Benetti, Giuseppe Legname
Oct 16, 2010·F1000 Biology Reports·Federico BenettiGiuseppe Legname
Mar 29, 2011·Nature Structural & Molecular Biology·Vytautas SmirnovasWitold K Surewicz
Oct 12, 2011·Proceedings of the National Academy of Sciences of the United States of America·Fred F DambergerKurt Wüthrich
Oct 12, 2012·Prion·Jingjing Liang, Qingzhong Kong
Nov 20, 2013·The Journal of Biological Chemistry·Alex J McDonaldGlenn L Millhauser
Feb 14, 2014·Redox Report : Communications in Free Radical Research·Barbara S Berlett, Rodney L Levine
Sep 4, 2014·Redox Biology·Renata L S GoncalvesMartin D Brand
Oct 5, 2014·Journal of Molecular Biology·Federico BenettiGiuseppe Legname

❮ Previous
Next ❯

Citations

Jan 14, 2018·The Journal of Biological Chemistry·Pavithra ChandramowlishwaranYury O Chernoff
Jul 26, 2019·International Journal of Molecular Sciences·Livia Bernardi, Amalia C Bruni
Oct 1, 2020·International Journal of Molecular Sciences·Hajar Miranzadeh Mahabadi, Changiz Taghibiglou

❮ Previous
Next ❯

Methods Mentioned

BETA
infrared spectroscopy
nuclear
X-ray
glycosylation
protein folding
protein
differential scanning calorimetry

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Journal of Molecular Biology
Federico BenettiGiuseppe Legname
Biomedicine & Pharmacotherapy = Biomédecine & Pharmacothérapie
S LehmannA Mangé
Anales de la Real Academia Nacional de Medicina
G Suárez Fernández
© 2022 Meta ULC. All rights reserved