Small heat shock proteins: Simplicity meets complexity

M Haslbeck, S Weinkauf, J Buchner - Journal of Biological Chemistry, 2019 - ASBMB
Small heat shock proteins (sHsps) are a ubiquitous and ancient family of ATP-independent
molecular chaperones. A key characteristic of sHsps is that they exist in ensembles of iso …

The chaperone-assisted selective autophagy complex dynamics and dysfunctions

B Tedesco, L Vendredy, V Timmerman, A Poletti - Autophagy, 2023 - Taylor & Francis
Each protein must be synthesized with the correct amino acid sequence, folded into its
native structure, and transported to a relevant subcellular location and protein complex. If …

Small heat shock proteins operate as molecular chaperones in the mitochondrial intermembrane space

E Adriaenssens, B Asselbergh, P Rivera-Mejías… - Nature Cell …, 2023 - nature.com
Mitochondria are complex organelles with different compartments, each harbouring their
own protein quality control factors. While chaperones of the mitochondrial matrix are well …

Heat-shock chaperone HSPB1 regulates cytoplasmic TDP-43 phase separation and liquid-to-gel transition

S Lu, J Hu, OA Arogundade, A Goginashvili… - Nature cell …, 2022 - nature.com
While acetylated, RNA-binding-deficient TDP-43 reversibly phase separates within nuclei
into complex droplets (anisosomes) comprised of TDP-43-containing liquid outer shells and …

A surveillance function of the HSPB8-BAG3-HSP70 chaperone complex ensures stress granule integrity and dynamism

M Ganassi, D Mateju, I Bigi, L Mediani, I Poser, HO Lee… - Molecular cell, 2016 - cell.com
Stress granules (SGs) are ribonucleoprotein complexes induced by stress. They sequester
mRNAs and disassemble when the stress subsides, allowing translation restoration. In …

ATP7A-related copper transport diseases—emerging concepts and future trends

SG Kaler - Nature reviews Neurology, 2011 - nature.com
This Review summarizes recent advances in understanding copper-transporting ATPase 1
(ATP7A), and examines the neurological phenotypes associated with dysfunction of this …

Modulation of neurodegeneration by molecular chaperones

PJ Muchowski, JL Wacker - Nature Reviews Neuroscience, 2005 - nature.com
Many neurodegenerative disorders are characterized by conformational changes in proteins
that result in misfolding, aggregation and intra-or extra-neuronal accumulation of amyloid …

Large potentials of small heat shock proteins

EV Mymrikov, AS Seit-Nebi… - Physiological …, 2011 - journals.physiology.org
Modern classification of the family of human small heat shock proteins (the so-called HSPB)
is presented, and the structure and properties of three members of this family are analyzed …

Role of axonal transport in neurodegenerative diseases

KJ De Vos, AJ Grierson, S Ackerley… - Annu. Rev …, 2008 - annualreviews.org
Many major human neurodegenerative diseases, including Alzheimer's disease,
Parkinson's disease, and amyotrophic lateral sclerosis (ALS), display axonal pathologies …

[HTML][HTML] Exome sequence analysis suggests that genetic burden contributes to phenotypic variability and complex neuropathy

C Gonzaga-Jauregui, T Harel, T Gambin, M Kousi… - Cell reports, 2015 - cell.com
Summary Charcot-Marie-Tooth (CMT) disease is a clinically and genetically heterogeneous
distal symmetric polyneuropathy. Whole-exome sequencing (WES) of 40 individuals from 37 …