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Stress granules, RNA-binding proteins and polyglutamine diseases: too much aggregation?
Stress granules (SGs) are membraneless cell compartments formed in response to different
stress stimuli, wherein translation factors, mRNAs, RNA-binding proteins (RBPs) and other …
stress stimuli, wherein translation factors, mRNAs, RNA-binding proteins (RBPs) and other …
RNA-binding proteins with prion-like domains in health and disease
AF Harrison, J Shorter - Biochemical Journal, 2017 - portlandpress.com
Approximately 70 human RNA-binding proteins (RBPs) contain a prion-like domain (PrLD).
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …
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 …
trinucleotide repeat in the gene encoding the huntingtin protein. Despite its well-defined …
A chaperome subnetwork safeguards proteostasis in aging and neurodegenerative disease
Chaperones are central to the proteostasis network (PN) and safeguard the proteome from
misfolding, aggregation, and proteotoxicity. We categorized the human chaperome of 332 …
misfolding, aggregation, and proteotoxicity. We categorized the human chaperome of 332 …
The life cycle of the 26S proteasome: from birth, through regulation and function, and onto its death
The 26S proteasome is a large,∼ 2.5 MDa, multi-catalytic ATP-dependent protease complex
that serves as the degrading arm of the ubiquitin system, which is the major pathway for …
that serves as the degrading arm of the ubiquitin system, which is the major pathway for …
Drosophila as an In Vivo Model for Human Neurodegenerative Disease
With the increase in the ageing population, neurodegenerative disease is devastating to
families and poses a huge burden on society. The brain and spinal cord are extraordinarily …
families and poses a huge burden on society. The brain and spinal cord are extraordinarily …
Cellular strategies for controlling protein aggregation
J Tyedmers, A Mogk, B Bukau - Nature reviews Molecular cell biology, 2010 - nature.com
The aggregation of misfolded proteins is associated with the perturbation of cellular function,
ageing and various human disorders. Mounting evidence suggests that protein aggregation …
ageing and various human disorders. Mounting evidence suggests that protein aggregation …
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 …
cause and pathological mechanism—the misfolding, aggregation and accumulation of …
Polyglutamine (PolyQ) Diseases: Navigating the Landscape of Neurodegeneration
Polyglutamine (polyQ) diseases are a group of inherited neurodegenerative disorders
caused by expanded cytosine-adenine-guanine (CAG) repeats encoding proteins with …
caused by expanded cytosine-adenine-guanine (CAG) repeats encoding proteins with …
Impairment of the ubiquitin-proteasome system by protein aggregation
NF Bence, RM Sampat, RR Kopito - Science, 2001 - science.org
Intracellular deposition of aggregated and ubiquitylated proteins is a prominent
cytopathological feature of most neurodegenerative disorders. Whether protein aggregates …
cytopathological feature of most neurodegenerative disorders. Whether protein aggregates …