Protein misfolding, functional amyloid, and human disease
Peptides or proteins convert under some conditions from their soluble forms into highly
ordered fibrillar aggregates. Such transitions can give rise to pathological conditions ranging …
ordered fibrillar aggregates. Such transitions can give rise to pathological conditions ranging …
Amyloid formation by globular proteins under native conditions
The conversion of proteins from their soluble states into well-organized fibrillar aggregates is
associated with a wide range of pathological conditions, including neurodegenerative …
associated with a wide range of pathological conditions, including neurodegenerative …
Molecular pathogenesis of spinocerebellar ataxias
AM Duenas, R Goold, P Giunti - Brain, 2006 - academic.oup.com
The autosomal dominant spinocerebellar ataxias (SCAs) are a group of neurodegenerative
diseases, clinically and genetically heterogeneous, characterized by loss of balance and …
diseases, clinically and genetically heterogeneous, characterized by loss of balance and …
How do ALS-associated mutations in superoxide dismutase 1 promote aggregation of the protein?
BF Shaw, JS Valentine - Trends in biochemical sciences, 2007 - cell.com
More than 100 different mutations in the gene encoding copper-zinc superoxide dismutase
(SOD1) cause familial forms of amyotrophic lateral sclerosis (ALS)–a fatal …
(SOD1) cause familial forms of amyotrophic lateral sclerosis (ALS)–a fatal …
Physical chemistry of polyglutamine: intriguing tales of a monotonous sequence
R Wetzel - Journal of molecular biology, 2012 - Elsevier
Polyglutamine (polyQ) sequences of unknown normal function are present in a significant
number of proteins, and their repeat expansion is associated with a number of genetic …
number of proteins, and their repeat expansion is associated with a number of genetic …
Functional insights from the distribution and role of homopeptide repeat-containing proteins
Expansion of “low complex” repeats of amino acids such as glutamine (Poly-Q) is associated
with protein misfolding and the development of degenerative diseases such as Huntington's …
with protein misfolding and the development of degenerative diseases such as Huntington's …
Polyglutamine diseases: the special case of ataxin-3 and Machado–Joseph disease
Polyglutamine (polyQ) diseases are a group of nine neurodegenerative disorders caused by
an unstable CAG expansion in the codifying region of their respective associated genes …
an unstable CAG expansion in the codifying region of their respective associated genes …
The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step
AM Ellisdon, B Thomas, SP Bottomley - Journal of Biological Chemistry, 2006 - ASBMB
The aggregation of ataxin-3 is associated with spinocerebellar ataxia type 3, which is
characterized by the formation of intraneuronal aggregates. However, the mechanism of …
characterized by the formation of intraneuronal aggregates. However, the mechanism of …
AFM-based single molecule techniques: unraveling the amyloid pathogenic species
FS Ruggeri, J Habchi, A Cerreta… - Current pharmaceutical …, 2016 - ingentaconnect.com
Background: A wide class of human diseases and neurodegenerative disorders, such as
Alzheimer's disease, is due to the failure of a specific peptide or protein to keep its native …
Alzheimer's disease, is due to the failure of a specific peptide or protein to keep its native …
Assays for α-synuclein aggregation
L Giehm, N Lorenzen, DE Otzen - Methods, 2011 - Elsevier
This review describes different ways to achieve and monitor reproducible aggregation of α-
synuclein, a key protein in the development of Parkinson's disease. For most globular …
synuclein, a key protein in the development of Parkinson's disease. For most globular …