Citation Impact
Citing Papers
TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
2008 StandoutScience
Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration
2013
Caenorhabditis elegans ADAR editing and the ERI-6/7/MOV10 RNAi pathway silence endogenous viral elements and LTR retrotransposons
2020 StandoutNobel
The C9orf72 protein interacts with Rab1a and the ULK 1 complex to regulate initiation of autophagy
2016
C9orf72 is required for proper macrophage and microglial function in mice
2016 Science
Strategy for Dual-Analyte Luciferin Imaging: In Vivo Bioluminescence Detection of Hydrogen Peroxide and Caspase Activity in a Murine Model of Acute Inflammation
2013 StandoutNobel
C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins
2014 Science
Induced Pluripotent Stem Cells Generated from Patients with ALS Can Be Differentiated into Motor Neurons
2008 StandoutScience
Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia
2006 StandoutScience
Consensus Statement of the Movement Disorder Society on Tremor
2008 Standout
Neurodegenerative disease mutations in TREM2 reveal a functional surface and distinct loss-of-function mechanisms
2016
Loss of C9 ORF 72 impairs autophagy and synergizes with polyQ Ataxin‐2 to induce motor neuron dysfunction and cell death
2016
Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy
2015
Update on the glutamatergic neurotransmitter system and the role of excitotoxicity in amyotrophic lateral sclerosis
2002
Altered microglial response to Aβ plaques in APPPS1-21 mice heterozygous for TREM2
2014
Diverse Brain Myeloid Expression Profiles Reveal Distinct Microglial Activation States and Aspects of Alzheimer’s Disease Not Evident in Mouse Models
2018
A split, conditionally active mimetic of IL-2 reduces the toxicity of systemic cytokine therapy
2022 StandoutNobel
Mutant Copper-Zinc Superoxide Dismutase (SOD1) Induces Protein Secretion Pathway Alterations and Exosome Release in Astrocytes
2013
TREM2 in CNS homeostasis and neurodegenerative disease
2015
Apolipoprotein E Is a Ligand for Triggering Receptor Expressed on Myeloid Cells 2 (TREM2)
2015
Human Endogenous Retroviruses in Neurological Diseases
2018
The role of FUS gene variants in neurodegenerative diseases
2014
PARP-1 dependent recruitment of the amyotrophic lateral sclerosis-associated protein FUS/TLS to sites of oxidative DNA damage
2013
Inflammatory processes in amyotrophic lateral sclerosis
2002
Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria
2004
Developmental neurotoxicity of industrial chemicals
2006 Standout
TREM2 in Neurodegenerative Diseases
2017
The Long-Term Outcome of Microvascular Decompression for Trigeminal Neuralgia
1996 Standout
Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease
2007
Improving Outcomes of Neuroprotection by Minocycline
2010
Alzheimer’s Disease Risk Genes and Mechanisms of Disease Pathogenesis
2014
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations
2013
Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS
2008
A single family with writer's cramp, essential tremor, and primary writing tremor
1987
Mechanisms Underlying Inflammation in Neurodegeneration
2010 Standout
Pathological roles of MAPK signaling pathways in human diseases
2010 Standout
CD22 blockade restores homeostatic microglial phagocytosis in ageing brains
2019 StandoutNatureNobel
TDP43-Positive Intraneuronal Inclusions in a Patient with Motor Neuron Disease and Parkinson’s Disease
2010
Aberrant RNA Processing in a Neurodegenerative Disease: the Cause for Absent EAAT2, a Glutamate Transporter, in Amyotrophic Lateral Sclerosis
1998
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration
2010
Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways
2015
Good practice in the management of amyotrophic lateral sclerosis: Clinical guidelines. An evidence‐based review with good practice points. EALSC Working Group
2007
Primary writing tremor. A form of focal dystonia?
1990
Programmable RNA Tracking in Live Cells with CRISPR/Cas9
2016 StandoutNobel
Self-propagation of pathogenic protein aggregates in neurodegenerative diseases
2013 Nature
TREM2 variants: new keys to decipher Alzheimer disease pathogenesis
2016
Consensus guidelines for the design and implementation of clinical trials in ALS
1999
A Neurodegeneration-Specific Gene-Expression Signature of Acutely Isolated Microglia from an Amyotrophic Lateral Sclerosis Mouse Model
2013
Clinical aspects of ALS in Gulf War Veterans
2008
Minocycline Inhibition of Monocyte Activation Correlates with Neuronal Protection in SIV NeuroAIDS
2011
The Role of Immune and Inflammatory Mechanisms in ALS
2011
C9orf72 nucleotide repeat structures initiate molecular cascades of disease
2014 Nature
Tremor and idiopathic dystonia
1991
Amyotrophic lateral sclerosis
2011 Standout
TRIBE: Hijacking an RNA-Editing Enzyme to Identify Cell-Specific Targets of RNA-Binding Proteins
2016 StandoutNobel
TAR-DNA Binding Protein 43 in Pick Disease
2008
Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis
2016
Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology
2013
The Enigmatic Role of C9ORF72 in Autophagy
2017
Cognitive impairment in amyotrophic lateral sclerosis
2007
Parkinson's disease
2009 Standout
The management of motor neurone disease
2003
Mitochondrial Myopathy Caused by Long-Term Zidovudine Therapy
1990 Standout
Cytokines and chemokines: At the crossroads of cell signalling and inflammatory disease
2014 Standout
Biological Functions of Autophagy Genes: A Disease Perspective
2019 Standout
Systemic dysregulation of TDP-43 binding microRNAs in amyotrophic lateral sclerosis
2013
TREM2 Lipid Sensing Sustains the Microglial Response in an Alzheimer’s Disease Model
2015 Standout
Chapter 1 The Molecular Links Between TDP‐43 Dysfunction and Neurodegeneration
2009
Decoding ALS: from genes to mechanism
2016 StandoutNature
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport
2015 Nature
Biomarkers in amyotrophic lateral sclerosis
2008
Systemic inflammatory cells fight off neurodegenerative disease
2010
Considerations and Challenges in Studying Liquid-Liquid Phase Separation and Biomolecular Condensates
2019 Standout
Mechanisms of minocycline-induced suppression of simian immunodeficiency virus encephalitis: inhibition of apoptosis signal-regulating kinase 1
2008
Alzheimer Disease: An Update on Pathobiology and Treatment Strategies
2019 Standout
Mutational analysis of the Cu/Zn superoxide dismutase gene in a Catalan ALS population: Should all sporadic ALS cases also be screened for SOD1?
2006
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia
2010
Distinct cerebral lesions in sporadic and ‘D90A’ SOD1 ALS: studies with [11C]flumazenil PET
2005
The executioners sing a new song: killer caspases activate microglia
2011
Reactive oxygen species trigger motoneuron death in non-cell-autonomous models of ALS through activation of c-Abl signaling
2015
RNA-binding proteins with prion-like domains in ALS and FTLD-U
2011
Modulating inflammatory monocytes with a unique microRNA gene signature ameliorates murine ALS
2012
Parkinson's disease
2015 Standout
Proton Magnetic Resonance Spectroscopy Reveals Neuroprotection by Oral Minocycline in a Nonhuman Primate Model of Accelerated NeuroAIDS
2010
Lipid-droplet-accumulating microglia represent a dysfunctional and proinflammatory state in the aging brain
2020 StandoutNobel
Misregulated RNA processing in amyotrophic lateral sclerosis
2012
Neuromuscular diseases associated with human immunodeficiency virus infection
1988
The immunology of stroke: from mechanisms to translation
2011 Standout
Amyotrophic Lateral Sclerosis-Associated SOD1 Mutant Proteins Bind and Aggregate with Bcl-2 in Spinal Cord Mitochondria
2004
Alzheimer's disease
2016 Standout
New insights into the role of TREM2 in Alzheimer’s disease
2018
Mouse Inoculation Studies Reveal No Transmissible Agent in Amyotrophic Lateral Sclerosis
1996
TDP-43 high throughput screening analyses in neurodegeneration: Advantages and pitfalls
2013
Microvascular decompression for hemifacial spasm
1995
TREM2 regulates microglial cell activation in response to demyelination in vivo
2015
The Triggering Receptor Expressed on Myeloid Cells 2 Binds Apolipoprotein E
2015
Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS
2014
Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis
2005
Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases
2006 StandoutNature
Overview and recent advances in neuropathology. Part 2: Neurodegeneration
2011
A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation
2015 Standout
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies
2009
The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease
2012
Cerebrospinal fluid soluble TREM2 is higher in Alzheimer disease and associated with mutation status
2016
The Amyloid State of Proteins in Human Diseases
2012 Standout
Physical activity as an exogenous risk factor in motor neuron disease (MND): A review of the evidence
2009
Regenerating and denervated human muscle fibers and satellite cells express neural cell adhesion molecule recognized by monoclonal antibodies to natural killer cells
1992
Three dimensions of the amyloid hypothesis: time, space and 'wingmen'
2015
State of play in amyotrophic lateral sclerosis genetics
2013
EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives
2005
Reduced C9orf72 protein levels in frontal cortex of amyotrophic lateral sclerosis and frontotemporal degeneration brain with the C9ORF72 hexanucleotide repeat expansion
2014
Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2–21.3
2006
Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent
1997 StandoutNature
Novel drug development for amyotrophic lateral sclerosis
2000
Molecular biology of amyotrophic lateral sclerosis: insights from genetics
2006
Polymyositis and dermatomyositis
2003 Standout
A Unique Microglia Type Associated with Restricting Development of Alzheimer’s Disease
2017 Standout
Minocycline and neurodegenerative diseases
2008
Phase Separation by Low Complexity Domains Promotes Stress Granule Assembly and Drives Pathological Fibrillization
2015 Standout
Single-cell transcriptomic analysis of Alzheimer’s disease
2019 StandoutNature
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
2008
Amyotrophic Lateral Sclerosis
2001 Standout
Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers
2015
Exosome secretion is a key pathway for clearance of pathological TDP-43
2016
Expression of Amyotrophic Lateral Sclerosis-linked SOD1 Mutant Increases the Neurotoxic Potential of Microglia via TLR2
2008
A Controlled Trial of High-Dose Intravenous Immune Globulin Infusions as Treatment for Dermatomyositis
1993 Standout
TREM2 deficiency reduces the efficacy of immunotherapeutic amyloid clearance
2016
Intermolecular transmission of superoxide dismutase 1 misfolding in living cells
2011
Neurodegenerative Diseases: Expanding the Prion Concept
2015
Amyotrophic lateral sclerosis
2000
Clinical Trials in Motor Neuron Diseases
1999
Stress granules as crucibles of ALS pathogenesis
2013
MicroRNAs as Biomarkers in Amyotrophic Lateral Sclerosis
2018
Minocycline treatment for HIV-associated cognitive impairment
2011
TREM2 deficiency eliminates TREM2+ inflammatory macrophages and ameliorates pathology in Alzheimer’s disease mouse models
2015
Copper Active Sites in Biology
2014 Standout
ALZHEIMER'S DISEASE, PARKINSON'S DISEASE, AND MOTONEURONE DISEASE: ABIOTROPIC INTERACTION BETWEEN AGEING AND ENVIRONMENT?
1986
Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models
2007
Copper Homeostasis and Neurodegenerative Disorders (Alzheimer's, Prion, and Parkinson's Diseases and Amyotrophic Lateral Sclerosis)
2006 Standout
Writing tremor after discrete cortical infarction.
1994
Microglia Function in the Central Nervous System During Health and Neurodegeneration
2017 Standout
DAMP-sensing receptors in sterile inflammation and inflammatory diseases
2019 Standout
Minocycline: far beyond an antibiotic
2013 Standout
Spermidine in health and disease
2018 StandoutScience
How neuroinflammation contributes to neurodegeneration
2016 StandoutScience
Glatiramer acetate in combination with minocycline in patients with relapsing—remitting multiple sclerosis: results of a Canadian, multicenter, double-blind, placebo-controlled trial
2009
Classification and Treatment of Tremor
1991
The biology , function , and biomedical applications of exosomes
2020 StandoutScience
Genotype-phenotype correlations between GBA mutations and Parkinson disease risk and onset
2008
Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: A population-based study.
2000
Immature Copper-Zinc Superoxide Dismutase and Familial Amyotrophic Lateral Sclerosis
2009
Excitotoxicity and Amyotrophic Lateral Sclerosis
2005
Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms
2014
Assessment of disease progression in motor neuron disease
2005
COPPER-ZINC SUPEROXIDE DISMUTASE AND AMYOTROPHIC LATERAL SCLEROSIS
2003
TREM2 sustains microglial expansion during aging and response to demyelination
2015
Metals in Neurobiology: Probing Their Chemistry and Biology with Molecular Imaging
2008 Standout
Extracellular microRNAs in human circulation are associated with miRISC complexes that are accessible to anti-AGO2 antibody and can bind target mimic oligonucleotides
2020 StandoutNobel
Works of John Ravits being referenced
Dysregulated mi RNA biogenesis downstream of cellular stress and ALS ‐causing mutations: a new mechanism for ALS
2015
Misfolded SOD1 is not a primary component of sporadic ALS
2017
Controversies and priorities in amyotrophic lateral sclerosis
2013
Botulinum toxin therapy in hemifacial spasm: Clinical and electrophysiologic studies
1989
TDP-43 and ubiquitinated cytoplasmic aggregates in sporadic ALS are low frequency and widely distributed in the lower motor neuron columns independent of disease spread
2010
Pathological TDP‐43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations
2007
Placebo-controlled trial of gabapentin in patients with amyotrophic lateral sclerosis
1996
Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial
2007
Sporadic ALS has compartment-specific aberrant exon splicing and altered cell–matrix adhesion biology
2009
Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: a decade of discoveries, defects and disputes
2003
A4V superoxide dismutase mutation in apparently sporadic ALS resembling neuralgic amyotrophy
2006
No evidence of HIV pol gene in spinal cord tissues in sporadic ALS by real-time RT-PCR
2010
Lack of C9ORF72 coding mutations supports a gain of function for repeat expansions in amyotrophic lateral sclerosis
2013
ALS motor phenotype heterogeneity, focality, and spread
2009
TREM2Variant p.R47H as a Risk Factor for Sporadic Amyotrophic Lateral Sclerosis
2014
Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS
2013
A Long-Term Follow-up Study of Patients with Post-Poliomyelitis Neuromuscular Symptoms
1986
Focality of upper and lower motor neuron degeneration at the clinical onset of ALS
2007
Implications of ALS focality
2007
Targeting RNA Foci in iPSC-Derived Motor Neurons from ALS Patients with a C9ORF72 Repeat Expansion
2013
Amyotrophic lateral sclerosis onset is influenced by the burden of rare variants in known amyotrophic lateral sclerosis genes
2014
Primary writing tremor and myoclonic writer's cramp
1985
Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: a decade of discoveries, defects and disputes
2003