Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms

TDP-43 and FUS en route from the nucleus to the cytoplasm

(2017) Helena Ederle et al.   FEBS LETTERS

TDP-43 and Cytoskeletal Proteins in ALS

(2017) Moritz Oberstadt et al.   MOLECULAR NEUROBIOLOGY

Enhancing Mitofusin/Marf ameliorates neuromuscular dysfunction in Drosophila models of TDP-43 proteinopathies

(2017) Bilal Khalil et al.   NEUROBIOLOGY OF AGING

Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments

(2017) Tesfaye W. Tefera et al.   Frontiers in Neuroscience

Inside out: the role of nucleocytoplasmic transport in ALS and FTLD

(2016) Steven Boeynaems et al.   ACTA NEUROPATHOLOGICA

Mitochondria‐associated membrane collapse is a common pathomechanism in SIGMAR1 ‐ and SOD1 ‐linked ALS

(2016) Seiji Watanabe et al.   EMBO Molecular Medicine

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Early and gender-specific differences in spinal cord mitochondrial function and oxidative stress markers in a mouse model of ALS

(2016) Daniel Cacabelos et al.   Acta Neuropathologica Communications

Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts

(2016) Elisa Onesto et al.   Acta Neuropathologica Communications

In vitro studies in VCP-associated multisystem proteinopathy suggest altered mitochondrial bioenergetics

(2015) Angèle Nalbandian et al.   MITOCHONDRION

Altered age-related changes in bioenergetic properties and mitochondrial morphology in fibroblasts from sporadic amyotrophic lateral sclerosis patients

(2015) Scott P. Allen et al.   NEUROBIOLOGY OF AGING

Overexpression of nuclear FUS induces neuronal cell death

(2015) H. Suzuki et al.   NEUROSCIENCE

Nonnative SOD1 trimer is toxic to motor neurons in a model of amyotrophic lateral sclerosis

(2015) Elizabeth A. Proctor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

FUS Interacts with HSP60 to Promote Mitochondrial Damage

(2015) Jianwen Deng et al.   PLoS Genetics

Mitochondria dynamism: of shape, transport and cell migration

(2014) André Ferreira da Silva et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Disturbed mitochondrial dynamics and neurodegenerative disorders

(2014) Florence Burté et al.   Nature Reviews Neurology

The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons

(2013) Wenzhang Wang et al.   HUMAN MOLECULAR GENETICS

Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

(2013) Jordi Magrané et al.   HUMAN MOLECULAR GENETICS

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

Altered Intracellular Localization of SOD1 in Leukocytes from Patients with Sporadic Amyotrophic Lateral Sclerosis

(2013) Cristina Cereda et al.   PLoS One

Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis

(2012) Gabriel E. Rodríguez et al.   ARQUIVOS DE NEURO-PSIQUIATRIA

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1α

(2012) Wenjun Song et al.   NEUROBIOLOGY OF DISEASE

An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1

(2012) S. Guareschi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial dynamics in neurodegeneration

(2012) Kie Itoh et al.   TRENDS IN CELL BIOLOGY

Protein Misdirection Inside and Outside Motor Neurons in Amyotrophic Lateral Sclerosis (ALS): A Possible Clue for Therapeutic Strategies

(2011) Akemi Ido et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Amyotrophic Lateral Sclerosis Multiprotein Biomarkers in Peripheral Blood Mononuclear Cells

(2011) Giovanni Nardo et al.   PLoS One

TDP-43 and FUS: a nuclear affair

(2011) Dorothee Dormann et al.   TRENDS IN NEUROSCIENCES

Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice

(2010) Y.-F. Xu et al.   JOURNAL OF NEUROSCIENCE

Mitochondrial dysfunction and intracellular calcium dysregulation in ALS

(2010) Hibiki Kawamata et al.   MECHANISMS OF AGEING AND DEVELOPMENT

SOD1 mRNA expression in sporadic amyotrophic lateral sclerosis

(2010) Stella Gagliardi et al.   NEUROBIOLOGY OF DISEASE

In Vivo Accumulation of Helicobacter pylori Products, NOD1, Ubiquitinated Proteins and Proteasome in a Novel Cytoplasmic Structure

(2010) Vittorio Necchi et al.   PLoS One

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

(2009) Hristelina Ilieva et al.   JOURNAL OF CELL BIOLOGY

Focal degeneration of astrocytes in amyotrophic lateral sclerosis

(2008) D Rossi et al.   CELL DEATH AND DIFFERENTIATION

Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity

(2008) Gabriella Dobrowolny et al.   Cell Metabolism

Fission and selective fusion govern mitochondrial segregation and elimination by autophagy

(2008) Gilad Twig et al.   EMBO JOURNAL