Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

Beclin 1 bridges autophagy, apoptosis and differentiation

(2014) Jianrong Wang   Autophagy

Does bafilomycin A1block the fusion of autophagosomes with lysosomes?

(2014) Daniel J. Klionsky et al.   Autophagy

Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration

(2013) Andrey S Tsvetkov et al.   Nature Chemical Biology

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

The Pathological Phenotypes of Human TDP-43 Transgenic Mouse Models Are Independent of Downregulation of Mouse Tdp-43

(2013) Ya-Fei Xu et al.   PLoS One

Translation of HTT mRNA with expanded CAG repeats is regulated by the MID1–PP2A protein complex

(2013) Sybille Krauß et al.   Nature Communications

Mouse models of frontotemporal dementia

(2012) Erik D. Roberson   ANNALS OF NEUROLOGY

Rodent models of TDP-43: Recent advances

(2012) William Tsao et al.   BRAIN RESEARCH

mTOR Signaling in Growth Control and Disease

(2012) Mathieu Laplante et al.   CELL

Accelerated Disease Onset with Stabilized Familial Amyotrophic Lateral Sclerosis (ALS)-linked Mutant TDP-43 Proteins

(2012) Shoji Watanabe et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability

(2012) B. Bilican et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43

(2012) I.-F. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 pathological changes in early onset familial and sporadic Alzheimer’s disease, late onset Alzheimer’s disease and Down’s Syndrome: association with age, hippocampal sclerosis and clinical phenotype

(2011) Yvonne S. Davidson et al.   ACTA NEUROPATHOLOGICA

Rapamycin treatment augments motor neuron degeneration in SOD1G93Amouse model of amyotrophic lateral sclerosis

(2011) Xiaojie Zhang et al.   Autophagy

The Human Brain in a Dish: The Promise of iPSC-Derived Neurons

(2011) Ricardo Dolmetsch et al.   CELL

Induction of autophagy with catalytic mTOR inhibitors reduces huntingtin aggregates in a neuronal cell model

(2011) Ana Roscic et al.   JOURNAL OF NEUROCHEMISTRY

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

(2011) Amanda M Haidet-Phillips et al.   NATURE BIOTECHNOLOGY

Pathogenic TARDBP Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: Disease-Associated Pathways

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Quantitative Relationships between Huntingtin Levels, Polyglutamine Length, Inclusion Body Formation, and Neuronal Death Provide Novel Insight into Huntington's Disease Molecular Pathogenesis

(2010) J. Miller et al.   JOURNAL OF NEUROSCIENCE

Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis

(2010) S. J. Barmada et al.   JOURNAL OF NEUROSCIENCE

TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor

(2010) C. M. Dewey et al.   MOLECULAR AND CELLULAR BIOLOGY

The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease

(2010) Jonathan H Fox et al.   Molecular Neurodegeneration

Bridging the Valley of Death of therapeutics for neurodegeneration

(2010) Steven Finkbeiner   NATURE MEDICINE

ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS

(2010) S.-C. Ling et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A small-molecule scaffold induces autophagy in primary neurons and protects against toxicity in a Huntington disease model

(2010) A. S. Tsvetkov et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Integration of Clearance Mechanisms: The Proteasome and Autophagy

(2010) E. Wong et al.   Cold Spring Harbor Perspectives in Biology

Clinical and Pathological Continuum of Multisystem TDP-43 Proteinopathies

(2009) Felix Geser et al.   ARCHIVES OF NEUROLOGY

Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL −/− mice: Support for a role for TDP-43 in the physiological response to neuronal injury

(2009) Katie Moisse et al.   BRAIN RESEARCH

XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

(2009) C. Hetz et al.   GENES & DEVELOPMENT

Astrocytic protection of spinal motor neurons but not cortical neurons against loss of Als2/alsin function

(2009) A. Jacquier et al.   HUMAN MOLECULAR GENETICS

Molecular Neuropathology of TDP-43 Proteinopathies

(2009) Manuela Neumann   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Degradation of TDP-43 and its pathogenic form by autophagy and the ubiquitin-proteasome system

(2009) Xiaoju Wang et al.   NEUROSCIENCE LETTERS

TDP-43A315T mutation in familial motor neuron disease

(2008) Michael A. Gitcho et al.   ANNALS OF NEUROLOGY

Structural determinants of the cellular localization and shuttling of TDP-43

(2008) Y. M. Ayala et al.   JOURNAL OF CELL SCIENCE

Aggregates Assembled From Overexpression of Wild-Type α-Synuclein are not Toxic to Human Neuronal Cells

(2008) Li-wen Ko et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY