User profiles for M. Stephani

Mariane Stefani

- Verified email at ufg.br - Cited by 3743

Marco Antonio Stefani

- Verified email at stefani.med.br - Cited by 3087

Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution

M Stefani, CM Dobson - Journal of molecular medicine, 2003 - Springer
The deposition of proteins in the form of amyloid fibrils and plaques is the characteristic feature
of more than 20 degenerative conditions affecting either the central nervous system or a …

[HTML][HTML] Plant selective autophagy—still an uncharted territory with a lot of hidden gems

M Stephani, Y Dagdas - Journal of Molecular Biology, 2020 - Elsevier
Selective autophagy has emerged as a major quality control pathway that surgically removes
damaged or unwanted macromolecules to maintain cellular health. Defects in selective …

[HTML][HTML] Protein misfolding and aggregation: new examples in medicine and biology of the dark side of the protein world

M Stefani - Biochimica et biophysica acta (BBA)-Molecular basis of …, 2004 - Elsevier
The data reported in the past 5 years have highlighted new aspects of protein misfolding
and aggregation. Firstly, it appears that protein aggregation may be a generic property of …

A cross-kingdom conserved ER-phagy receptor maintains endoplasmic reticulum homeostasis during stress

M Stephani, L Picchianti, A Gajic, R Beveridge… - elife, 2020 - elifesciences.org
Eukaryotes have evolved various quality control mechanisms to promote proteostasis in the
endoplasmic reticulum (ER). Selective removal of certain ER domains via autophagy (…

Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases

…, J Zurdo, N Taddei, G Ramponi, CM Dobson, M Stefani - nature, 2002 - nature.com
A range of human degenerative conditions, including Alzheimer's disease, light-chain
amyloidosis and the spongiform encephalopathies, is associated with the deposition in tissue of …

Designing conditions for in vitro formation of amyloid protofilaments and fibrils

…, N Taddei, A Clark, M Stefani… - Proceedings of the …, 1999 - National Acad Sciences
We have been able to convert a small α/β protein, acylphosphatase, from its soluble and
native form into insoluble amyloid fibrils of the type observed in a range of pathological …

Rationalization of the effects of mutations on peptide andprotein aggregation rates

F Chiti, M Stefani, N Taddei, G Ramponi, CM Dobson - Nature, 2003 - nature.com
In order for any biological system to function effectively, it is essential to avoid the inherent
tendency of proteins to aggregate and form potentially harmful deposits 1 , 2 , 3 , 4 . In each of …

Medical management with or without interventional therapy for unruptured brain arteriovenous malformations (ARUBA): a multicentre, non-blinded, randomised trial

…, WL Young, E Houdart, C Cordonnier, MA Stefani… - The Lancet, 2014 - thelancet.com
Background The clinical benefit of preventive eradication of unruptured brain arteriovenous
malformations remains uncertain. A Randomised trial of Unruptured Brain Arteriovenous …

Shuffled ATG8 interacting motifs form an ancestral bridge between UFMylation and autophagy

…, N Zhan, NAT Irwin, R Groh, M Stephani… - The EMBO …, 2023 - embopress.org
UFMylation involves the covalent modification of substrate proteins with UFM1 (Ubiquitin‐fold
modifier 1) and is important for maintaining ER homeostasis. Stalled translation triggers …

A causative link between the structure of aberrant protein oligomers and their toxicity

…, C Parrini, E Evangelisti, A Relini, M Stefani… - Nature chemical …, 2010 - nature.com
The aberrant assembly of peptides and proteins into fibrillar aggregates proceeds through
oligomeric intermediates that are thought to be the primary pathogenic species in many …