The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease

OD King, AD Gitler, J Shorter - Brain research, 2012 - Elsevier
Prions are self-templating protein conformers that are naturally transmitted between
individuals and promote phenotypic change. In yeast, prion-encoded phenotypes can be …

Use of Caenorhabditis elegans as a model to study Alzheimer's disease and other neurodegenerative diseases

AG Alexander, V Marfil, C Li - Frontiers in genetics, 2014 - frontiersin.org
Advances in research and technology has increased our quality of life, allowed us to combat
diseases, and achieve increased longevity. Unfortunately, increased longevity is …

Human Hsp70 disaggregase reverses Parkinson's-linked α-synuclein amyloid fibrils

X Gao, M Carroni, C Nussbaum-Krammer, A Mogk… - Molecular cell, 2015 - cell.com
Intracellular amyloid fibrils linked to neurodegenerative disease typically accumulate in an
age-related manner, suggesting inherent cellular capacity for counteracting amyloid …

Cellular strategies of protein quality control

B Chen, M Retzlaff, T Roos… - Cold Spring Harbor …, 2011 - cshperspectives.cshlp.org
Eukaryotic cells must contend with a continuous stream of misfolded proteins that
compromise the cellular protein homeostasis balance and jeopardize cell viability. An …

Aging as an event of proteostasis collapse

RC Taylor, A Dillin - Cold Spring Harbor perspectives in …, 2011 - cshperspectives.cshlp.org
Aging cells accumulate damaged and misfolded proteins through a functional decline in
their protein homeostasis (proteostasis) machinery, leading to reduced cellular viability and …

The mammalian disaggregase machinery: Hsp110 synergizes with Hsp70 and Hsp40 to catalyze protein disaggregation and reactivation in a cell-free system

J Shorter - PloS one, 2011 - journals.plos.org
Bacteria, fungi, protozoa, chromista and plants all harbor homologues of Hsp104, a AAA+
ATPase that collaborates with Hsp70 and Hsp40 to promote protein disaggregation and …

Metazoan Hsp70 machines use Hsp110 to power protein disaggregation

H Rampelt, J Kirstein‐Miles, NB Nillegoda, K Chi… - The EMBO …, 2012 - embopress.org
Accumulation of aggregation‐prone misfolded proteins disrupts normal cellular function and
promotes ageing and disease. Bacteria, fungi and plants counteract this by solubilizing and …

Protein rescue from aggregates by powerful molecular chaperone machines

SM Doyle, O Genest, S Wickner - Nature reviews Molecular cell biology, 2013 - nature.com
Protein quality control within the cell requires the interplay of many molecular chaperones
and proteases. When this quality control system is disrupted, polypeptides follow pathways …

Small heat shock proteins potentiate amyloid dissolution by protein disaggregases from yeast and humans

ML Duennwald, AL Echeverria, J Shorter - PLoS biology, 2012 - journals.plos.org
How small heat shock proteins (sHsps) might empower proteostasis networks to control
beneficial prions or disassemble pathological amyloid is unknown. Here, we establish that …

[HTML][HTML] Cellular strategies for regulating functional and nonfunctional protein aggregation

J Gsponer, MM Babu - Cell reports, 2012 - cell.com
Growing evidence suggests that aggregation-prone proteins are both harmful and functional
for a cell. How do cellular systems balance the detrimental and beneficial effect of protein …