Multiple roles for the cytoskeleton in ALS

Biochemical characterization of actin assembly mechanisms with ALS-associated profilin variants

(2022) Xinbei Liu et al.   EUROPEAN JOURNAL OF CELL BIOLOGY

ALS-associated KIF5A mutations abolish autoinhibition resulting in a toxic gain of function

(2022) Desiree M. Baron et al.   Cell Reports

C9orf72-Derived Proline:Arginine Poly-Dipeptides Modulate Cytoskeleton and Mechanical Stress Response

(2022) Tomo Shiota et al.   Frontiers in Cell and Developmental Biology

Visualizing molecules of functional human profilin

(2022) Morgan L Pimm et al.   eLife

A Pictorial History of the Neuronal Cytoskeleton

(2021) Christophe Leterrier   JOURNAL OF NEUROSCIENCE

Self-repair protects microtubules from destruction by molecular motors

(2021) Sarah Triclin et al.   NATURE MATERIALS

New twists in actin–microtubule interactions

(2021) Morgan L. Pimm et al.   MOLECULAR BIOLOGY OF THE CELL

Gene co-expression network analysis in human spinal cord highlights mechanisms underlying amyotrophic lateral sclerosis susceptibility

(2021) Jerry C. Wang et al.   Scientific Reports

Cryo‐EM reveals the complex architecture of dynactin's shoulder region and pointed end

(2021) Clinton K Lau et al.   EMBO JOURNAL

C9orf72-derived arginine-containing dipeptide repeats associate with axonal transport machinery and impede microtubule-based motility

(2021) Laura Fumagalli et al.   Science Advances

A CRMP4‐dependent retrograde axon‐to‐soma death signal in amyotrophic lateral sclerosis

(2021) Roy Maimon et al.   EMBO JOURNAL

Highly accurate protein structure prediction with AlphaFold

(2021) John Jumper et al.   NATURE

ALS-linked PFN1 variants exhibit loss and gain of functions in the context of formin-induced actin polymerization

(2021) Eric J. Schmidt et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Profilin and Mical combine to impair F-actin assembly and promote disassembly and remodeling

(2021) Elena E. Grintsevich et al.   Nature Communications

C9orf72 ALS/FTD dipeptide repeat protein levels are reduced by small molecules that inhibit PKA or enhance protein degradation

(2021) Nausicaa V Licata et al.   EMBO JOURNAL

New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis

(2021) Frances Theunissen et al.   Translational Neurodegeneration

Breaking of buckled microtubules is mediated by kinesins

(2020) Arif Md. Rashedul Kabir et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Intermediate filaments in developing neurons: Beyond structure

(2020) Christopher J. Bott et al.   Cytoskeleton

Specific depletion of the motor protein KIF5B leads to deficits in dendritic transport, synaptic plasticity and memory

(2020) Junjun Zhao et al.   eLife

Much More Than a Scaffold: Cytoskeletal Proteins in Neurological Disorders

(2020) Diana C. Muñoz-Lasso et al.   Cells

Arp2/3 and Mena/VASP Require Profilin 1 for Actin Network Assembly at the Leading Edge

(2020) Kristen Skruber et al.   CURRENT BIOLOGY

Profilin 1 delivery tunes cytoskeletal dynamics toward CNS axon regeneration

(2020) Rita Pinto-Costa et al.   JOURNAL OF CLINICAL INVESTIGATION

Amyotrophic lateral sclerosis: a clinical review

(2020) P. Masrori et al.   EUROPEAN JOURNAL OF NEUROLOGY

Loss of Tdp-43 disrupts the axonal transcriptome of motoneurons accompanied by impaired axonal translation and mitochondria function

(2020) Michael Briese et al.   Acta Neuropathologica Communications

Neurofilaments in motor neuron disorders: towards promising diagnostic and prognostic biomarkers

(2020) Elisabetta Zucchi et al.   Molecular Neurodegeneration

Spastin depletion increases tubulin polyglutamylation and impairs kinesin-mediated neuronal transport, leading to working and associative memory deficits

(2020) André T. Lopes et al.   PLOS BIOLOGY

Microtubule self-repair

(2020) Manuel Théry et al.   CURRENT OPINION IN CELL BIOLOGY

Diagnostic and Prognostic Performance of Neurofilaments in ALS

(2019) Koen Poesen et al.   Frontiers in Neurology

Disrupted neuronal trafficking in amyotrophic lateral sclerosis

(2019) Katja Burk et al.   ACTA NEUROPATHOLOGICA

The role of neurofilament aggregation in neurodegeneration: lessons from rare inherited neurological disorders

(2019) Alessandro Didonna et al.   Molecular Neurodegeneration

Dynactin1 depletion leads to neuromuscular synapse instability and functional abnormalities

(2019) Valérie Bercier et al.   Molecular Neurodegeneration

An allosteric network in spastin couples multiple activities required for microtubule severing

(2019) Colby R. Sandate et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Whole-exome sequencing identified novel KIF5A mutations in Chinese patients with amyotrophic lateral sclerosis and Charcot-Marie-Tooth type 2

(2019) Jing He et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Motor Neuron Susceptibility in ALS/FTD

(2019) Audrey M. G. Ragagnin et al.   Frontiers in Neuroscience

Role of actin in organelle trafficking in neurons

(2019) Keertana Venkatesh et al.   Cytoskeleton

Structure of spastin bound to a glutamate-rich peptide implies a hand-over-hand mechanism of substrate translocation

(2019) Han Han et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Modulation of actin polymerization affects nucleocytoplasmic transport in multiple forms of amyotrophic lateral sclerosis

(2019) Anthony Giampetruzzi et al.   Nature Communications

Changes to the TDP-43 and FUS Interactomes Induced by DNA Damage

(2019) Tetsuya Kawaguchi et al.   JOURNAL OF PROTEOME RESEARCH

Hot-spot KIF5A mutations cause familial ALS

(2018) David Brenner et al.   BRAIN

Reconsidering an active role for G-actin in cytoskeletal regulation

(2018) Kristen Skruber et al.   JOURNAL OF CELL SCIENCE

Genome-wide Analyses Identify KIF5A as a Novel ALS Gene

(2018) Aude Nicolas et al.   NEURON

ALS Genes in the Genomic Era and their Implications for FTD

(2018) Hung Phuoc Nguyen et al.   TRENDS IN GENETICS

TDP-43 self-interaction is modulated by redox-active compounds Auranofin, Chelerythrine and Riluzole

(2018) Moritz Oberstadt et al.   Scientific Reports

Rab5 and Alsin regulate stress-activated cytoprotective signaling on mitochondria

(2018) FoSheng Hsu et al.   eLife

Altered oligomeric states in pathogenic ALS2 variants associated with juvenile motor neuron diseases cause loss of ALS2-mediated endosomal function

(2018) Kai Sato et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutation analysis of KIF5A in Chinese amyotrophic lateral sclerosis patients

(2018) Kang Zhang et al.   NEUROBIOLOGY OF AGING

ALS-causing mutations in profilin-1 alter its conformational dynamics: A computational approach to explain propensity for aggregation

(2018) Mahmoud Kiaei et al.   Scientific Reports

Actin–microtubule crosstalk in cell biology

(2018) Marileen Dogterom et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

FUS inclusions disrupt RNA localization by sequestering kinesin-1 and inhibiting microtubule detyrosination

(2017) Kyota Yasuda et al.   JOURNAL OF CELL BIOLOGY

Tubulin isoform composition tunes microtubule dynamics

(2017) Annapurna Vemu et al.   MOLECULAR BIOLOGY OF THE CELL

TDP-43 and Cytoskeletal Proteins in ALS

(2017) Moritz Oberstadt et al.   MOLECULAR NEUROBIOLOGY

The impact of cytoskeletal organization on the local regulation of neuronal transport

(2017) Jeffrey J. Nirschl et al.   NATURE REVIEWS NEUROSCIENCE

Differentiation between Oppositely Oriented Microtubules Controls Polarized Neuronal Transport

(2017) Roderick P. Tas et al.   NEURON

Dendritic Actin Cytoskeleton: Structure, Functions, and Regulations

(2017) Anja Konietzny et al.   Frontiers in Cellular Neuroscience

Neurofilaments and Neurofilament Proteins in Health and Disease

(2017) Aidong Yuan et al.   Cold Spring Harbor Perspectives in Biology

TDP-43 misexpression causes defects in dendritic growth

(2017) Josiah J. Herzog et al.   Scientific Reports

Ginsenoside Re Attenuates Neuroinflammation in a Symptomatic ALS Animal Model

(2016) Mudan Cai et al.   AMERICAN JOURNAL OF CHINESE MEDICINE

Biophysical analysis of three novel profilin-1 variants associated with amyotrophic lateral sclerosis indicates a correlation between their aggregation propensity and the structural features of their globular state

(2016) Edoardo Del Poggetto et al.   BIOLOGICAL CHEMISTRY

Microtubule Defects Influence Kinesin-Based Transport In Vitro

(2016) Winnie H. Liang et al.   BIOPHYSICAL JOURNAL

Absence of alsin function leads to corticospinal motor neuron vulnerability via novel disease mechanisms

(2016) Mukesh Gautam et al.   HUMAN MOLECULAR GENETICS

Familial Amyotrophic Lateral Sclerosis-linked Mutations inProfilin 1Exacerbate TDP-43-induced Degeneration in the Retina ofDrosophila melanogasterthrough an Increase in the Cytoplasmic Localization of TDP-43

(2016) Koji Matsukawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

C9ORF72 interaction with cofilin modulates actin dynamics in motor neurons

(2016) Rajeeve Sivadasan et al.   NATURE NEUROSCIENCE

Internetwork competition for monomers governs actin cytoskeleton organization

(2016) Cristian Suarez et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Profilin 1 mutants form aggregates that induce accumulation of prion-like TDP-43

(2016) Yoshinori Tanaka et al.   Prion

Neuronal profilins in health and disease: Relevance for spine plasticity and Fragile X syndrome

(2016) Kristin Michaelsen-Preusse et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Actin and Actin-Binding Proteins

(2016) Thomas D. Pollard   Cold Spring Harbor Perspectives in Biology

Coordinating Neuronal Actin–Microtubule Dynamics

(2015) Charlotte H. Coles et al.   CURRENT BIOLOGY

X-ray and Cryo-EM structures reveal mutual conformational changes of Kinesin and GTP-state microtubules upon binding

(2015) M. Morikawa et al.   EMBO JOURNAL

TUBA4A gene analysis in sporadic amyotrophic lateral sclerosis: identification of novel mutations

(2015) Viviana Pensato et al.   JOURNAL OF NEUROLOGY

Molecular wear of microtubules propelled by surface-adhered kinesins

(2015) Emmanuel L. P. Dumont et al.   Nature Nanotechnology

Building the Neuronal Microtubule Cytoskeleton

(2015) Lukas C. Kapitein et al.   NEURON

Structural basis for mutation-induced destabilization of profilin 1 in ALS

(2015) Sivakumar Boopathy et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways

(2015) E. T. Cirulli et al.   SCIENCE

Golgi fragmentation in amyotrophic lateral sclerosis, an overview of possible triggers and consequences

(2015) Vinod Sundaramoorthy et al.   Frontiers in Neuroscience

Amyotrophic lateral sclerosis onset is influenced by the burden of rare variants in known amyotrophic lateral sclerosis genes

(2014) Janet Cady et al.   ANNALS OF NEUROLOGY

Glial cells in amyotrophic lateral sclerosis

(2014) T. Philips et al.   EXPERIMENTAL NEUROLOGY

Unique Function of Kinesin Kif5A in Localization of Mitochondria in Axons

(2014) P. D. Campbell et al.   JOURNAL OF NEUROSCIENCE

Profilin 1 Associates with Stress Granules and ALS-Linked Mutations Alter Stress Granule Dynamics

(2014) M. D. Figley et al.   JOURNAL OF NEUROSCIENCE

Exome-wide Rare Variant Analysis Identifies TUBA4A Mutations Associated with Familial ALS

(2014) Bradley N. Smith et al.   NEURON

Unbiased screen reveals ubiquilin-1 and -2 highly associated with huntingtin inclusions

(2013) Nicola J. Rutherford et al.   BRAIN RESEARCH

Functions of Class V Myosins in Neurons

(2013) John A. Hammer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Analysis of the Human Tissue-specific Expression by Genome-wide Integration of Transcriptomics and Antibody-based Proteomics

(2013) Linn Fagerberg et al.   MOLECULAR & CELLULAR PROTEOMICS

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

Axonal transport deficits and neurodegenerative diseases

(2013) Stéphanie Millecamps et al.   NATURE REVIEWS NEUROSCIENCE

Dynactin Deficiency in the CNS of Humans with Sporadic ALS and Mice with Genetically Determined Motor Neuron Degeneration

(2013) Magdalena Kuźma-Kozakiewicz et al.   NEUROCHEMICAL RESEARCH

Intricate interplay between astrocytes and motor neurons in ALS

(2013) H. P. Phatnani et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The dynactin p150 subunit: cell biology studies of sequence changes found in ALS/MND and Parkinsonian Syndromes

(2012) Marianne Stockmann et al.   JOURNAL OF NEURAL TRANSMISSION

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

(2012) Chi-Hong Wu et al.   NATURE

Screening of the PFN1 gene in sporadic amyotrophic lateral sclerosis and in frontotemporal dementia

(2012) Cinzia Tiloca et al.   NEUROBIOLOGY OF AGING

A novel phosphorylation site mutation in profilin 1 revealed in a large screen of US, Nordic, and German amyotrophic lateral sclerosis/frontotemporal dementia cohorts

(2012) Caroline Ingre et al.   NEUROBIOLOGY OF AGING

Phosphorylation-regulated axonal dependent transport of syntaxin 1 is mediated by a Kinesin-1 adapter

(2012) J. J. E. Chua et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Effects of ALS-related SOD1 mutants on dynein- and KIF5-mediated retrograde and anterograde axonal transport

(2010) Ping Shi et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

HDAC6 controls autophagosome maturation essential for ubiquitin-selective quality-control autophagy

(2010) Joo-Yong Lee et al.   EMBO JOURNAL

SOD1, ANG, VAPB, TARDBP, and FUS mutations in familial amyotrophic lateral sclerosis: genotype-phenotype correlations

(2010) S. Millecamps et al.   JOURNAL OF MEDICAL GENETICS

A novel peripherin gene (PRPH) mutation identified in one sporadic amyotrophic lateral sclerosis patient

(2010) Lucia Corrado et al.   NEUROBIOLOGY OF AGING

Differentiation of Hereditary Spastic Paraparesis From Primary Lateral Sclerosis in Sporadic Adult-Onset Upper Motor Neuron Syndromes

(2009) Frans Brugman et al.   ARCHIVES OF NEUROLOGY

Opposite-polarity motors activate one another to trigger cargo transport in live cells

(2009) Shabeen Ally et al.   JOURNAL OF CELL BIOLOGY

Myosin-dependent targeting of transmembrane proteins to neuronal dendrites

(2009) Tommy L Lewis et al.   NATURE NEUROSCIENCE

Neurodegeneration mutations in dynactin impair dynein-dependent nuclear migration

(2009) J. K. Moore et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Regulation of endosomal motility and degradation by amyotrophic lateral sclerosis 2/alsin

(2009) Chen Lai et al.   Molecular Brain

Heterozygous S44L missense change of the spastin gene in amyotrophic lateral sclerosis

(2008) Christoph Münch et al.   Amyotrophic Lateral Sclerosis

Quantitative and Functional Analyses of Spastin in the Nervous System: Implications for Hereditary Spastic Paraplegia

(2008) J. M. Solowska et al.   JOURNAL OF NEUROSCIENCE

Structural basis of microtubule severing by the hereditary spastic paraplegia protein spastin

(2008) Antonina Roll-Mecak et al.   NATURE