Toward understanding Machado–Joseph disease

M do Carmo Costa, HL Paulson - Progress in neurobiology, 2012 - Elsevier
Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is
the most common inherited spinocerebellar ataxia and one of many polyglutamine …

Protein aggregation: mechanisms and functional consequences

G Invernizzi, E Papaleo, R Sabate, S Ventura - The international journal of …, 2012 - Elsevier
Understanding the mechanisms underlying protein misfolding and aggregation has become
a central issue in biology and medicine. Compelling evidence show that the formation of …

Paclitaxel is incorporated by mesenchymal stromal cells and released in exosomes that inhibit in vitro tumor growth: a new approach for drug delivery

L Pascucci, V Coccè, A Bonomi, D Ami… - Journal of controlled …, 2014 - Elsevier
Mesenchymal stromal cells (MSCs) have been proposed for delivering anticancer agents
because of their ability to home in on tumor microenvironment. We found that MSCs can …

[HTML][HTML] Infrared nanospectroscopy characterization of oligomeric and fibrillar aggregates during amyloid formation

FS Ruggeri, G Longo, S Faggiano, E Lipiec… - Nature …, 2015 - nature.com
Amyloids are insoluble protein fibrillar aggregates. The importance of characterizing their
aggregation has steadily increased because of their link to human diseases and material …

Slow amyloid nucleation via α-helix-rich oligomeric intermediates in short polyglutamine-containing huntingtin fragments

M Jayaraman, R Kodali, B Sahoo, AK Thakur… - Journal of molecular …, 2012 - Elsevier
The 17-amino-acid N-terminal segment (httNT) that leads into the polyglutamine (polyQ)
segment in the Huntington's disease protein huntingtin (htt) dramatically increases …

Molecular interaction between the chaperone Hsc70 and the N-terminal flank of huntingtin exon 1 modulates aggregation

E Monsellier, V Redeker, G Ruiz-Arlandis… - Journal of Biological …, 2015 - ASBMB
The aggregation of polyglutamine (polyQ)-containing proteins is at the origin of nine
neurodegenerative diseases. Molecular chaperones prevent the aggregation of polyQ …

Wheat gliadin/xanthan gum intermolecular complexes: Interaction mechanism and structural characterization

M Avadanei, CE Brunchi - Food Chemistry, 2024 - Elsevier
In this study, the interactions between wheat gliadin (GL) and xanthan gum (XG) were
investigated to design new systems with potential applications as a gluten-free substitute …

Insights into the aggregation mechanism of PolyQ proteins with different glutamine repeat lengths

T Yushchenko, E Deuerling, K Hauser - Biophysical journal, 2018 - cell.com
Polyglutamine (polyQ) diseases, including Huntington's disease, result from the aggregation
of an abnormally expanded polyQ repeat in the affected protein. The length of the polyQ …

The hydrogen-bonding ability of the amino acid glutamine revealed by neutron diffraction experiments

NH Rhys, AK Soper, L Dougan - The journal of physical chemistry …, 2012 - ACS Publications
Hydrogen bonding between glutamine residues has been identified as playing an important
role in the intermolecular association and aggregation of proteins. To establish the …

Monomeric, oligomeric and polymeric proteins in huntington disease and other diseases of polyglutamine expansion

G Hoffner, P Djian - Brain sciences, 2014 - mdpi.com
Huntington disease and other diseases of polyglutamine expansion are each caused by a
different protein bearing an excessively long polyglutamine sequence and are associated …