Implications of peptide assemblies in amyloid diseases

PC Ke, MA Sani, F Ding, A Kakinen, I Javed… - Chemical Society …, 2017 - pubs.rsc.org
Neurodegenerative disorders and type 2 diabetes are global epidemics compromising the
quality of life of millions worldwide, with profound social and economic implications. Despite …

[HTML][HTML] Passive immunotherapies targeting Aβ and tau in Alzheimer's disease

SS Plotkin, NR Cashman - Neurobiology of Disease, 2020 - Elsevier
Amyloid-β (Aβ) and tau proteins currently represent the two most promising targets to treat
Alzheimer's disease. The most extensively developed method to treat the pathologic forms of …

Polyproline-II helix in proteins: structure and function

AA Adzhubei, MJE Sternberg, AA Makarov - Journal of molecular biology, 2013 - Elsevier
The poly-l-proline type II (PPII) helix in recent years has emerged clearly as a structural class
not only of fibrillar proteins (in collagen, PPII is a dominant conformation) but also of the …

Mammalian prion biology: one century of evolving concepts

A Aguzzi, M Polymenidou - Cell, 2004 - cell.com
Prions have been responsible for an entire century of tragic episodes. Fifty years ago, kuru
decimated the population of Papua New Guinea. Then, iatrogenic transmission of prions …

Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS

A Israelson, N Arbel, S Da Cruz, H Ilieva, K Yamanaka… - Neuron, 2010 - cell.com
Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), a
neurodegenerative disease characterized by loss of motor neurons. With conformation …

Prevalence of lymphoreticular prion protein accumulation in UK tissue samples

DA Hilton, AC Ghani, L Conyers… - The Journal of …, 2004 - Wiley Online Library
This study aims to provide an estimate of the number of individuals in the UK who may be
incubating variant Creutzfeldt‐Jakob disease and at risk of causing iatrogenic spread of the …

Parallel in-register intermolecular β-sheet architectures for prion-seeded prion protein (PrP) amyloids

BR Groveman, MA Dolan, LM Taubner, A Kraus… - Journal of Biological …, 2014 - ASBMB
Structures of the infectious form of prion protein (eg PrP Sc or PrP-Scrapie) remain poorly
defined. The prevalent structural models of PrP Sc retain most of the native α-helices of the …

Identifying polyglutamine protein species in situ that best predict neurodegeneration

J Miller, M Arrasate, E Brooks, CP Libeu… - Nature chemical …, 2011 - nature.com
Polyglutamine (polyQ) stretches exceeding a threshold length confer a toxic function to
proteins that contain them and cause at least nine neurological disorders. The basis for this …

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria

C Vande Velde, TM Miller… - Proceedings of the …, 2008 - National Acad Sciences
Mutations in copper/zinc superoxide dismutase (SOD1) are causative for dominantly
inherited amyotrophic lateral sclerosis (ALS). Despite high variability in biochemical …

Transmissible spongiform encephalopathies

SJ Collins, VA Lawson, CL Masters - The Lancet, 2004 - thelancet.com
Nosologically, transmissible spongiform encephalopathies (TSE or prion diseases) should
be grouped with other neurodegenerative disorders such as Alzheimer's and Parkinson's …