The chaperone HSPB8 reduces the accumulation of truncated TDP-43 species in cells and protects against TDP-43-mediated toxicity

Transcriptional induction of the heat shock protein B8 mediates the clearance of misfolded proteins responsible for motor neuron diseases

(2016) Valeria Crippa et al.   Scientific Reports

Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death

(2015) Shangxi Xiao et al.   ACTA NEUROPATHOLOGICA

Specific protein homeostatic functions of small heat-shock proteins increase lifespan

(2015) Michel J. Vos et al.   AGING CELL

TDP-35 sequesters TDP-43 into cytoplasmic inclusions through binding with RNA

(2015) Mei-Xia Che et al.   FEBS LETTERS

The Role of the Protein Quality Control System in SBMA

(2015) Paola Rusmini et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Motoneuronal and muscle-selective removal of ALS-related misfolded proteins: Figure 1

(2015) Valeria Crippa et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Synergic prodegradative activity of Bicalutamide and trehalose on the mutant androgen receptor responsible for spinal and bulbar muscular atrophy

(2014) E. Giorgetti et al.   HUMAN MOLECULAR GENETICS

Metabolism and mis-metabolism of the neuropathological signature protein TDP-43

(2014) C.-C. Huang et al.   JOURNAL OF CELL SCIENCE

The amyloid state and its association with protein misfolding diseases

(2014) Tuomas P. J. Knowles et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

Clearance of the mutant androgen receptor in motoneuronal models of spinal and bulbar muscular atrophy

(2013) Paola Rusmini et al.   NEUROBIOLOGY OF AGING

Different anti-aggregation and pro-degradative functions of the members of the mammalian sHSP family in neurological disorders

(2013) S. Carra et al.   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES

Differential autophagy power in the spinal cord and muscle of transgenic ALS mice

(2013) Valeria Crippa et al.   Frontiers in Cellular Neuroscience

Guidelines for the use and interpretation of assays for monitoring autophagy

(2012) Daniel J. Klionsky et al.   Autophagy

Human Axonal Survival of Motor Neuron (a-SMN) Protein Stimulates Axon Growth, Cell Motility, C-C Motif Ligand 2 (CCL2), and Insulin-like Growth Factor-1 (IGF1) Production

(2012) Denise Locatelli et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Spinal muscular atrophy pathogenic mutations impair the axonogenic properties of axonal-survival of motor neuron

(2012) Denise Locatelli et al.   JOURNAL OF NEUROCHEMISTRY

The Aggregation and Neurotoxicity of TDP-43 and Its ALS-Associated 25 kDa Fragment Are Differentially Affected by Molecular Chaperones in Drosophila

(2012) Jenna M. Gregory et al.   PLoS One

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond

(2011) Sandrine Da Cruz et al.   CURRENT OPINION IN NEUROBIOLOGY

BAG3 mediates chaperone-based aggresome-targeting and selective autophagy of misfolded proteins

(2011) Martin Gamerdinger et al.   EMBO REPORTS

The Frontotemporal Syndromes of ALS. Clinicopathological Correlates

(2011) Michael Joseph Strong et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Muscle cells and motoneurons differentially remove mutant SOD1 causing familial amyotrophic lateral sclerosis

(2011) Elisa Onesto et al.   JOURNAL OF NEUROCHEMISTRY

Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration

(2011) Edward B. Lee et al.   NATURE REVIEWS NEUROSCIENCE

The anabolic/androgenic steroid nandrolone exacerbates gene expression modifications induced by mutant SOD1 in muscles of mice models of amyotrophic lateral sclerosis

(2011) Mariarita Galbiati et al.   PHARMACOLOGICAL RESEARCH

Alteration of protein folding and degradation in motor neuron diseases: Implications and protective functions of small heat shock proteins

(2011) Serena Carra et al.   PROGRESS IN NEUROBIOLOGY

Inhibition of α-synuclein aggregation by small heat shock proteins

(2011) Ilona B. Bruinsma et al.   PROTEINS-STRUCTURE FUNCTION AND BIOINFORMATICS

A role of small heat shock protein B8 (HspB8) in the autophagic removal of misfolded proteins responsible for neurodegenerative diseases

(2010) Valeria Crippa et al.   Autophagy

Emerging role of Alzheimer's disease-associated ubiquilin-1 in protein aggregation: Figure 1

(2010) Annakaisa Haapasalo et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

(2010) C. Lagier-Tourenne et al.   HUMAN MOLECULAR GENETICS

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)

(2010) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

Identification of theDrosophilaOrtholog of HSPB8

(2010) Serena Carra et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43 Mediates Degeneration in a Novel Drosophila Model of Disease Caused by Mutations in VCP/p97

(2010) G. P. Ritson et al.   JOURNAL OF NEUROSCIENCE

A DNAJB Chaperone Subfamily with HDAC-Dependent Activities Suppresses Toxic Protein Aggregation

(2010) Jurre Hageman et al.   MOLECULAR CELL

Proteasomal and autophagic degradative activities in spinal and bulbar muscular atrophy

(2010) Paola Rusmini et al.   NEUROBIOLOGY OF DISEASE

17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy

(2010) Paola Rusmini et al.   NEUROBIOLOGY OF DISEASE

Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

(2010) Felix Geser et al.   NEUROPATHOLOGY

TDP-43-Mediated Neuron Loss In Vivo Requires RNA-Binding Activity

(2010) Aaron Voigt et al.   PLoS One

In search of an “autophagomometer”

(2009) David C. Rubinsztein et al.   Autophagy

The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS)

(2009) Michael J. Strong   JOURNAL OF THE NEUROLOGICAL SCIENCES

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

(2009) Y.-J. Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A presynaptically toxic secreted phospholipase A2 is internalized into motoneuron-like cells where it is rapidly translocated into the cytosol

(2008) Zala Jenko Pražnikar et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation

(2008) Matthew J. Winton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

Vesicle associated membrane protein B (VAPB) is decreased in ALS spinal cord

(2008) Georgia Anagnostou et al.   NEUROBIOLOGY OF AGING

Mutations in the HSP27 (HSPB1) gene cause dominant, recessive, and sporadic distal HMN/CMT type 2

(2008) H. Houlden et al.   NEUROLOGY

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity

(2008) B. S. Johnson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis

(2008) Nicola J. Rutherford et al.   PLoS Genetics