Microglial activation in an amyotrophic lateral sclerosis-like model caused by Ranbp2 loss and nucleocytoplasmic transport impairment in retinal ganglion neurons

Repopulating retinal microglia restore endogenous organization and function under CX3CL1-CX3CR1 regulation

(2018) Yikui Zhang et al.   Science Advances

Matrix metalloproteinase 28 is regulated by TRIF- and type I IFN-dependent signaling in macrophages

(2018) Matthew E. Long et al.   Innate Immunity

Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer’s Disease

(2018) Bahareh Eftekharzadeh et al.   NEURON

Regulatory T cells promote myelin regeneration in the central nervous system

(2017) Yvonne Dombrowski et al.   NATURE NEUROSCIENCE

Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport

(2017) Fatima Gasset-Rosa et al.   NEURON

Mutant Huntingtin Disrupts the Nuclear Pore Complex

(2017) Jonathan C. Grima et al.   NEURON

Aberrant distributions of nuclear pore complex proteins in ALS mice and ALS patients

(2017) Jingwei Shang et al.   NEUROSCIENCE

Immunomodulation-accelerated neuronal regeneration following selective rod photoreceptor cell ablation in the zebrafish retina

(2017) David T. White et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ALS Along the Axons – Expression of Coding and Noncoding RNA Differs in Axons of ALS models

(2017) Nimrod Rotem et al.   Scientific Reports

Schwann cells and their transcriptional network: Evolution of key regulators of peripheral myelination

(2016) C. Claus Stolt et al.   BRAIN RESEARCH

Progranulin promotes peripheral nerve regeneration and reinnervation: role of notch signaling

(2016) Christine Altmann et al.   Molecular Neurodegeneration

Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System

(2016) Fernando J. Martinez et al.   NEURON

Mammalian-Specific Central Myelin Protein Opalin Is Redundant for Normal Myelination: Structural and Behavioral Assessments

(2016) Fumio Yoshikawa et al.   PLoS One

C9orf72 is required for proper macrophage and microglial function in mice

(2016) J. G. ORourke et al.   SCIENCE

Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses

(2016) Katannya Kapeli et al.   Nature Communications

The RanBP2/RanGAP1*SUMO1/Ubc9 SUMO E3 ligase is a disassembly machine for Crm1-dependent nuclear export complexes

(2016) Tobias Ritterhoff et al.   Nature Communications

Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD

(2016) Steven Boeynaems et al.   Scientific Reports

Macular sub-layer thinning and association with pulmonary function tests in Amyotrophic Lateral Sclerosis

(2016) Joseph M. Simonett et al.   Scientific Reports

Targeting the Cyclophilin Domain of Ran-binding Protein 2 (Ranbp2) with Novel Small Molecules to Control the Proteostasis of STAT3, hnRNPA2B1 and M-Opsin

(2015) Kyoung-in Cho et al.   ACS Chemical Neuroscience

Isoform-specific antibodies reveal distinct subcellular localizations of C9orf72 in amyotrophic lateral sclerosis

(2015) Shangxi Xiao et al.   ANNALS OF NEUROLOGY

Combined HDAC1 and HDAC2 Depletion Promotes Retinal Ganglion Cell Survival After Injury Through Reduction of p53 Target Gene Expression

(2015) Frédéric Lebrun-Julien et al.   ASN Neuro

Microglial phagocytosis of living photoreceptors contributes to inherited retinal degeneration

(2015) L. Zhao et al.   EMBO Molecular Medicine

Ablation of the Ferroptosis Inhibitor Glutathione Peroxidase 4 in Neurons Results in Rapid Motor Neuron Degeneration and Paralysis

(2015) Liuji Chen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Retinal involvement in amyotrophic lateral sclerosis: a study with optical coherence tomography and diffusion tensor imaging

(2015) Annemarie Hübers et al.   JOURNAL OF NEURAL TRANSMISSION

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

(2015) Brian D. Freibaum et al.   NATURE

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA

(2015) A. C. Woerner et al.   SCIENCE

Deleterious mutations in the essential mRNA metabolism factor, hGle1, in amyotrophic lateral sclerosis

(2014) Hannah M. Kaneb et al.   HUMAN MOLECULAR GENETICS

Differential Loss of Prolyl Isomerase or Chaperone Activity of Ran-binding Protein 2 (Ranbp2) Unveils Distinct Physiological Roles of Its Cyclophilin Domain in Proteostasis

(2014) Kyoung-in Cho et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Selective Impairment of a Subset of Ran-GTP-binding Domains of Ran-binding Protein 2 (Ranbp2) Suffices to Recapitulate the Degeneration of the Retinal Pigment Epithelium (RPE) Triggered byRanbp2Ablation

(2014) Hemangi Patil et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD

(2014) Michael E. Ward et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Regulator of Fatty Acid Metabolism, Acetyl Coenzyme A Carboxylase 1, Controls T Cell Immunity

(2014) J. Lee et al.   JOURNAL OF IMMUNOLOGY

TLR-driven early glycolytic reprogramming via the kinases TBK1-IKKɛ supports the anabolic demands of dendritic cell activation

(2014) Bart Everts et al.   NATURE IMMUNOLOGY

De novo fatty acid synthesis controls the fate between regulatory T and T helper 17 cells

(2014) Luciana Berod et al.   NATURE MEDICINE

Microglia and brain macrophages in the molecular age: from origin to neuropsychiatric disease

(2014) Marco Prinz et al.   NATURE REVIEWS NEUROSCIENCE

Neuronal Matrix Metalloproteinase-9 Is a Determinant of Selective Neurodegeneration

(2014) Artem Kaplan et al.   NEURON

RanBP2/Nup358 Potentiates the Translation of a Subset of mRNAs Encoding Secretory Proteins

(2014) Kohila Mahadevan et al.   PLOS BIOLOGY

Subtle retinal pathology in amyotrophic lateral sclerosis

(2014) Marius Ringelstein et al.   Annals of Clinical and Translational Neurology

Impaired Cytoplasmic-Nuclear Transport of Hypoxia-Inducible Factor-1α in Amyotrophic Lateral Sclerosis

(2013) Yuko Nagara et al.   BRAIN PATHOLOGY

Cellular Fatty Acid Metabolism and Cancer

(2013) Erin Currie et al.   Cell Metabolism

Cellular therapy to target neuroinflammation in amyotrophic lateral sclerosis

(2013) Federica Rizzo et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

MMP28 promotes macrophage polarization toward M2 cells and augments pulmonary fibrosis

(2013) S. A. Gharib et al.   JOURNAL OF LEUKOCYTE BIOLOGY

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Molecular signature of disease onset in Granulin mutation carriers: a gene expression analysis study

(2013) Elena Milanesi et al.   NEUROBIOLOGY OF AGING

Cholinergic modulation of response gain in the rat primary visual cortex

(2013) Shogo Soma et al.   Scientific Reports

A Neurodegeneration-Specific Gene-Expression Signature of Acutely Isolated Microglia from an Amyotrophic Lateral Sclerosis Mouse Model

(2013) Isaac M. Chiu et al.   Cell Reports

Kinesin-1 and mitochondrial motility control by discrimination of structurally equivalent but distinct subdomains in Ran-GTP-binding domains of Ran-binding protein 2

(2013) H. Patil et al.   Open Biology

Distinct and Atypical Intrinsic and Extrinsic Cell Death Pathways between Photoreceptor Cell Types upon Specific Ablation of Ranbp2 in Cone Photoreceptors

(2013) Kyoung-in Cho et al.   PLoS Genetics

Unravelling the enigma of selective vulnerability in neurodegeneration: motor neurons resistant to degeneration in ALS show distinct gene expression characteristics and decreased susceptibility to excitotoxicity

(2012) Alice Brockington et al.   ACTA NEUROPATHOLOGICA

Ranbp2 haploinsufficiency mediates distinct cellular and biochemical phenotypes in brain and retinal dopaminergic and glia cells elicited by the Parkinsonian neurotoxin, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)

(2012) Kyoung-in Cho et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

(2012) Clotilde Lagier-Tourenne et al.   NATURE NEUROSCIENCE

Differential gene and transcript expression analysis of RNA-seq experiments with TopHat and Cufflinks

(2012) Cole Trapnell et al.   Nature Protocols

The Oncogene eIF4E Reprograms the Nuclear Pore Complex to Promote mRNA Export and Oncogenic Transformation

(2012) Biljana Culjkovic-Kraljacic et al.   Cell Reports

Specific deficits in visual electrophysiology in a mouse model of dominant optic atrophy

(2011) Alun R. Barnard et al.   EXPERIMENTAL EYE RESEARCH

Age-related changes in visual function in cystathionine-beta-synthase mutant mice, a model of hyperhomocysteinemia

(2011) Minzhong Yu et al.   EXPERIMENTAL EYE RESEARCH

Ran-dependent docking of importin-β to RanBP2/Nup358 filaments is essential for protein import and cell viability

(2011) Masakazu Hamada et al.   JOURNAL OF CELL BIOLOGY

Novel optineurin mutations in patients with familial and sporadic amyotrophic lateral sclerosis

(2011) R. Del Bo et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

PTTG1/securin modulates microtubule nucleation and cell migration

(2011) Miguel A. Moreno-Mateos et al.   MOLECULAR BIOLOGY OF THE CELL

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

(2011) Magdalini Polymenidou et al.   NATURE NEUROSCIENCE

Physiology of Microglia

(2011) Helmut Kettenmann et al.   PHYSIOLOGICAL REVIEWS

The Nucleoporin Nup358/RanBP2 Promotes Nuclear Import in a Cargo- and Transport Receptor-Specific Manner

(2011) Sarah Wälde et al.   TRAFFIC

Neuroprotection resulting from insufficiency of RANBP2 is associated with the modulation of protein and lipid homeostasis of functionally diverse but linked pathways in response to oxidative stress

(2010) K.-i. Cho et al.   Disease Models & Mechanisms

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

(2010) Dorothee Dormann et al.   EMBO JOURNAL

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

DRR drives brain cancer invasion by regulating cytoskeletal-focal adhesion dynamics

(2010) P U Le et al.   ONCOGENE

Electrodiagnostic assessment in optic nerve disease

(2009) Graham E Holder et al.   CURRENT OPINION IN NEUROLOGY

RANBP2 is an allosteric activator of the conventional kinesin-1 motor protein, KIF5B, in a minimal cell-free system

(2009) Kyoung-in Cho et al.   EMBO REPORTS

Epilysin (MMP-28) Restrains Early Macrophage Recruitment in Pseudomonas aeruginosa Pneumonia

(2009) A. M. Manicone et al.   JOURNAL OF IMMUNOLOGY

Transcriptional targets for pituitary tumor-transforming gene-1

(2009) Yunguang Tong et al.   JOURNAL OF MOLECULAR ENDOCRINOLOGY

Nuclear Contour Irregularity and Abnormal Transporter Protein Distribution in Anterior Horn Cells in Amyotrophic Lateral Sclerosis

(2009) Yoshimi Kinoshita et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Stretch growth of integrated axon tracts: Extremes and exploitations

(2009) Douglas H. Smith   PROGRESS IN NEUROBIOLOGY

HDAC-class II specific inhibition involves HDAC proteasome-dependent degradation mediated by RANBP2

(2008) Annamaria Scognamiglio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

MMP-28 as a regulator of myelination

(2008) Sean R Werner et al.   BMC NEUROSCIENCE

Resolution of Sister Centromeres Requires RanBP2-Mediated SUMOylation of Topoisomerase IIα

(2008) Meelad M. Dawlaty et al.   CELL

Haploinsufficiency of RanBP2 is neuroprotective against light-elicited and age-dependent degeneration of photoreceptor neurons

(2008) K-in Cho et al.   CELL DEATH AND DIFFERENTIATION

Opalin, a Transmembrane Sialylglycoprotein Located in the Central Nervous System Myelin Paranodal Loop Membrane

(2008) Fumio Yoshikawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Transcriptome Database for Astrocytes, Neurons, and Oligodendrocytes: A New Resource for Understanding Brain Development and Function

(2008) J. D. Cahoy et al.   JOURNAL OF NEUROSCIENCE

Single-neuron labeling with inducible Cre-mediated knockout in transgenic mice

(2008) Paul Young et al.   NATURE NEUROSCIENCE