Huntington's disease: mechanisms of pathogenesis and therapeutic strategies

M Jimenez-Sanchez, F Licitra… - Cold Spring …, 2017 - perspectivesinmedicine.cshlp.org
Huntington's disease is a late-onset neurodegenerative disease caused by a CAG
trinucleotide repeat in the gene encoding the huntingtin protein. Despite its well-defined …

Protein aggregation and neurodegenerative disease

CA Ross, MA Poirier - Nature medicine, 2004 - nature.com
Neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD),
Huntington's disease (HD), amyotrophic lateral sclerosis (ALS) and prion diseases are …

Molecular mechanisms and potential therapeutical targets in Huntington's disease

C Zuccato, M Valenza, E Cattaneo - Physiological reviews, 2010 - journals.physiology.org
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG repeat
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …

Unfolding the role of protein misfolding in neurodegenerative diseases

C Soto - Nature Reviews Neuroscience, 2003 - nature.com
Recent evidence indicates that diverse neurodegenerative diseases might have a common
cause and pathological mechanism—the misfolding, aggregation and accumulation of …

Physical stability of proteins in aqueous solution: mechanism and driving forces in nonnative protein aggregation

EY Chi, S Krishnan, TW Randolph… - Pharmaceutical …, 2003 - Springer
Irreversible protein aggregation is problematic in the biotechnology industry, where
aggregation is encountered throughout the lifetime of a therapeutic protein, including during …

Effects of in vivo conditions on amyloid aggregation

MC Owen, D Gnutt, M Gao, SKTS Wärmländer… - Chemical Society …, 2019 - pubs.rsc.org
One of the grand challenges of biophysical chemistry is to understand the principles that
govern protein misfolding and aggregation, which is a highly complex process that is …

Techniques to study amyloid fibril formation in vitro

MR Nilsson - Methods, 2004 - Elsevier
Amyloid fibrils are ordered aggregates of peptides or proteins that are fibrillar in structure
and contribute to the complications of many diseases (eg, type 2 diabetes mellitus …

Functional amyloid formation within mammalian tissue

DM Fowler, AV Koulov, C Alory-Jost, MS Marks… - PLoS …, 2006 - journals.plos.org
Amyloid is a generally insoluble, fibrous cross-β sheet protein aggregate. The process of
amyloidogenesis is associated with a variety of neurodegenerative diseases including …

Conformational constraints for amyloid fibrillation: the importance of being unfolded

VN Uversky, AL Fink - Biochimica et Biophysica Acta (BBA)-Proteins and …, 2004 - Elsevier
Recent reports give strong support to the idea that amyloid fibril formation and the
subsequent development of protein deposition diseases originate from conformational …

Polyglutamine (PolyQ) Diseases: Navigating the Landscape of Neurodegeneration

R Tenchov, JM Sasso, QA Zhou - ACS Chemical Neuroscience, 2024 - ACS Publications
Polyglutamine (polyQ) diseases are a group of inherited neurodegenerative disorders
caused by expanded cytosine-adenine-guanine (CAG) repeats encoding proteins with …