Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS

Selective autophagy degrades nuclear pore complexes

(2020) Chia-Wei Lee et al.   NATURE CELL BIOLOGY

TORC1 inactivation stimulates autophagy of nucleoporin and nuclear pore complexes

(2020) Yui Tomioka et al.   JOURNAL OF CELL BIOLOGY

CHMPions of repair: Emerging perspectives on sensing and repairing the nuclear envelope barrier

(2020) C. Patrick Lusk et al.   CURRENT OPINION IN CELL BIOLOGY

Unrestrained ESCRT-III drives micronuclear catastrophe and chromosome fragmentation

(2020) Marina Vietri et al.   NATURE CELL BIOLOGY

G4C2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD

(2020) Alyssa N. Coyne et al.   NEURON

Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia

(2020) Mercedes Prudencio et al.   JOURNAL OF CLINICAL INVESTIGATION

The role of TDP-43 mislocalization in amyotrophic lateral sclerosis

(2020) Terry R. Suk et al.   Molecular Neurodegeneration

ALS Genetics: Gains, Losses, and Implications for Future Therapies

(2020) Garam Kim et al.   NEURON

ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair

(2019) Joseph R. Klim et al.   NATURE NEUROSCIENCE

Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration

(2019) Ze’ev Melamed et al.   NATURE NEUROSCIENCE

Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity

(2019) Yong-Jie Zhang et al.   SCIENCE

Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy

(2019) Jeannie Chew et al.   Molecular Neurodegeneration

Using induced pluripotent stem cell neuronal models to study neurodegenerative diseases

(2019) Xinwen Zhang et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

An ESCRT-LEM protein surveillance system is poised to directly monitor the nuclear envelope and nuclear transport system

(2019) David J Thaller et al.   eLife

Nucleocytoplasmic transport defects in neurodegeneration — Cause or consequence?

(2019) Saskia Hutten et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

The neuropathological diagnosis of Alzheimer’s disease

(2019) Michael A. DeTure et al.   Molecular Neurodegeneration

Nuclear pores as versatile reference standards for quantitative superresolution microscopy

(2019) Jervis Vermal Thevathasan et al.   NATURE METHODS

The many functions of ESCRTs

(2019) Marina Vietri et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Neurodegenerative disease concomitant proteinopathies are prevalent, age-related and APOE4-associated

(2018) John L Robinson et al.   BRAIN

Pathomechanisms of TDP-43 in neurodegeneration

(2018) Ju Gao et al.   JOURNAL OF NEUROCHEMISTRY

Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis

(2018) Yong-Jie Zhang et al.   NATURE MEDICINE

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

(2018) Ching-Chieh Chou et al.   NATURE NEUROSCIENCE

Evaluation of TDP-43 proteinopathy and hippocampal sclerosis in relation to APOE ε4 haplotype status: a community-based cohort study

(2018) Hyun-Sik Yang et al.   LANCET NEUROLOGY

Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer’s Disease

(2018) Bahareh Eftekharzadeh et al.   NEURON

Structures, Functions, and Dynamics of ESCRT-III/Vps4 Membrane Remodeling and Fission Complexes

(2018) John McCullough et al.   Annual Review of Cell and Developmental Biology

Visualization of long-lived proteins reveals age mosaicism within nuclei of postmitotic cells

(2018) Brandon H. Toyama et al.   JOURNAL OF CELL BIOLOGY

Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage

(2018) Sai Sampath Thammisetty et al.   Journal of Neuroinflammation

Association of Apolipoprotein E ε4 With Transactive Response DNA-Binding Protein 43

(2018) Alexandra M. Wennberg et al.   JAMA Neurology

Antisense Oligonucleotides: Translation from Mouse Models to Human Neurodegenerative Diseases

(2017) Kathleen M. Schoch et al.   NEURON

Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases

(2017) Hong Joo Kim et al.   NEURON

Mutant Huntingtin Disrupts the Nuclear Pore Complex

(2017) Jonathan C. Grima et al.   NEURON

LEM2 recruits CHMP7 for ESCRT-mediated nuclear envelope closure in fission yeast and human cells

(2017) Mingyu Gu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72 -associated amyotrophic lateral sclerosis

(2017) Tania F. Gendron et al.   Science Translational Medicine

Failure to Deliver and Translate—New Insights into RNA Dysregulation in ALS

(2017) Alyssa N. Coyne et al.   Frontiers in Cellular Neuroscience

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

(2016) Steven Boeynaems et al.   ACTA NEUROPATHOLOGICA

Timing and significance of pathological features inC9orf72expansion-associated frontotemporal dementia

(2016) Sarat C. Vatsavayai et al.   BRAIN

Chm7 and Heh1 collaborate to link nuclear pore complex quality control with nuclear envelope sealing

(2016) Brant M Webster et al.   EMBO JOURNAL

Nuclear transport dysfunction: a common theme in amyotrophic lateral sclerosis and frontotemporal dementia

(2016) Ana Jovičić et al.   JOURNAL OF NEUROCHEMISTRY

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Modeling ALS with motor neurons derived from human induced pluripotent stem cells

(2016) Samuel Sances et al.   NATURE NEUROSCIENCE

C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins

(2016) Yong-Jie Zhang et al.   NATURE NEUROSCIENCE

The nuclear pore complex: understanding its function through structural insight

(2016) Martin Beck et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The Role of TDP-43 in Alzheimer’s Disease

(2015) Xiao-Long Chang et al.   MOLECULAR NEUROBIOLOGY

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

TDP-43 is a key player in the clinical features associated with Alzheimer’s disease

(2014) Keith A. Josephs et al.   ACTA NEUROPATHOLOGICA

Surveillance of Nuclear Pore Complex Assembly by ESCRT-III/Vps4

(2014) Brant M. Webster et al.   CELL

Seeing the forest tree by tree: super-resolution light microscopy meets the neurosciences

(2013) Marta Maglione et al.   NATURE NEUROSCIENCE

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention

(2013) Christopher J. Donnelly et al.   NEURON

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Antisense Oligonucleotides: Treating Neurodegeneration at the Level of RNA

(2013) Sarah L. DeVos et al.   Neurotherapeutics

Nuclear pore complex composition: a new regulator of tissue-specific and developmental functions

(2012) Marcela Raices et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

ApoE and TDP-43 neuropathology in two siblings with familial FTLD-motor neuron disease

(2012) Keith A. Vossel et al.   NEUROCASE

Acute and chronically increased immunoreactivity to phosphorylation-independent but not pathological TDP-43 after a single traumatic brain injury in humans

(2011) Victoria E. Johnson et al.   ACTA NEUROPATHOLOGICA

The Structure of the Nuclear Pore Complex

(2011) André Hoelz et al.   Annual Review of Biochemistry

Multiple reprobing of Western blots after inactivation of peroxidase activity by its substrate, hydrogen peroxide

(2009) Alexis D. Sennepin et al.   ANALYTICAL BIOCHEMISTRY

Subdiffraction Multicolor Imaging of the Nuclear Periphery with 3D Structured Illumination Microscopy

(2008) L. Schermelleh et al.   SCIENCE