Standout Papers
Citation Impact
Citing Papers
Activating Mutations of NOTCH1 in Human T Cell Acute Lymphoblastic Leukemia
2004 StandoutScience
TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
2008 StandoutScience
In vivo imaging of hydrogen peroxide production in a murine tumor model with a chemoselective bioluminescent reporter
2010 StandoutNobel
α-Synuclein Promotes SNARE-Complex Assembly in Vivo and in Vitro
2010 StandoutScienceNobel
MicroRNA-206 Delays ALS Progression and Promotes Regeneration of Neuromuscular Synapses in Mice
2009 Science
Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice
2003 Science
Parkinson-like syndrome induced by continuous MPTP infusion: Convergent roles of the ubiquitin-proteasome system and α-synuclein
2005 StandoutNobel
Lithium delays progression of amyotrophic lateral sclerosis
2008
Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia
2006 StandoutScience
Dysregulated mi RNA biogenesis downstream of cellular stress and ALS ‐causing mutations: a new mechanism for ALS
2015
Alzheimer's disease cybrids replicate ?-amyloid abnormalities through cell death pathways
2000
Assembly of the γ-Secretase Complex Involves Early Formation of an Intermediate Subcomplex of Aph-1 and Nicastrin
2003
Neurotoxic Mechanisms Caused by the Alzheimer's Disease-linked Swedish Amyloid Precursor Protein Mutation
2003
Heparan sulfate regulates amyloid precursor protein processing by BACE1, the Alzheimer's β-secretase
2003
Advances in the Cellular and Molecular Biology of the Beta-Amyloid Protein in Alzheimer 's Disease
2002
No correlation between aggregates of Cu/Zn superoxide dismutase and cell death in familial amyotrophic lateral sclerosis
2002
Proteasomal inhibition causes the formation of protein aggregates containing a wide range of proteins, including nitrated proteins
2003
Therapeutic strategies for Alzheimer's disease
2002
Apoptosis in neurodegenerative disorders
2000
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
1995
Parkin Facilitates the Elimination of Expanded Polyglutamine Proteins and Leads to Preservation of Proteasome Function
2003
Astrocytes in amyotrophic lateral sclerosis: direct effects on motor neuron survival
2009
Nicastrin Functions as a γ-Secretase-Substrate Receptor
2005 StandoutNobel
Liposome-delivered superoxide dismutase prevents nitric oxide–dependent motor neuron death induced by trophic factor withdrawal
2000
Persistent activation of p38 mitogen-activated protein kinase in a mouse model of familial amyotrophic lateral sclerosis correlates with disease progression
2003
The Ubiquitin Proteasome System in Neurodegenerative Diseases
2003 StandoutNobel
Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1
2002
Linkage of a commoner form of recessive amyotrophic lateral sclerosis to chromosome 15q15-q22 markers
1998
Calcium dynamics and buffering in oculomotor neurones from mouse that are particularly resistant during amyotrophic lateral sclerosis (ALS)‐related motoneurone disease
2000
Inactivation of human Cu,Zn superoxide dismutase by peroxynitrite and formation of histidinyl radical
2004
Proteolytic Processing of Amyloid-β Precursor Protein by Secretases Does Not Require Cell Surface Transport
2004 StandoutNobel
Ubiquitin, cellular inclusions and their role in neurodegeneration
1998
SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model
2008
Follicular Dendritic Cells and Germinal Centers
1996
Formation and Removal of α-Synuclein Aggregates in Cells Exposed to Mitochondrial Inhibitors
2002
Minocycline delays disease onset and mortality in a transgenic model of ALS
2002
Upregulation of protein-tyrosine nitration in the anterior horn cells of amyotrophic lateral sclerosis
1997
Proprioceptive Sensory Neuropathy in Mice with a Mutation in the Cytoplasmic Dynein Heavy Chain 1 Gene
2007
CD22 blockade restores homeostatic microglial phagocytosis in ageing brains
2019 StandoutNatureNobel
Oxidative Stress Hypothesis in Alzheimer's Disease
1997 Standout
Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis
2013
SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter
1999
Abnormal diffusion tensor in nonsymptomatic familial amyotrophic lateral sclerosis with a causative superoxide dismutase 1 mutation
2007
GSK-3α regulates production of Alzheimer's disease amyloid-β peptides
2003 Nature
Enhancing Mitochondrial Calcium Buffering Capacity Reduces Aggregation of Misfolded SOD1 and Motor Neuron Cell Death without Extending Survival in Mouse Models of Inherited Amyotrophic Lateral Sclerosis
2013
Differentially expressed genes in sporadic amyotrophic lateral sclerosis spinal cords – screening by molecular indexing and subsequent cDNA microarray analysis
2002
Glutamate, excitotoxicity and amyotrophic lateral sclerosis
1997
The genetic and molecular mechanisms of motor neuron disease
1998
Superoxide dismutase and the death of motoneurons in ALS
2001
Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways
2015
Pathogenesis of two axonopathies does not require axonal neurofilaments
1998 Nature
Increased 3‐nitrotyrosine and oxidative damage in mice with a human copper/zinc superoxide dismutase mutation
1997
Oxidative Stress in Neurodegenerative Diseases
1996
Microglia Induce Motor Neuron Death via the Classical NF-κB Pathway in Amyotrophic Lateral Sclerosis
2014
TMP21 is a presenilin complex component that modulates γ-secretase but not ɛ-secretase activity
2006 Nature
Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model
2005
The Cell Adhesion Protein P-selectin Glycoprotein Ligand-1 Is a Substrate for the Aspartyl Protease BACE1
2003
Inactivation of Conserved C. elegans Genes Engages Pathogen- and Xenobiotic-Associated Defenses
2012 StandoutNobel
Inducible Nitric Oxide Synthase (iNOS) and Nitrotyrosine Immunoreactivity in the Spinal Cords of Transgenic Mice with a G93A MutantSOD1Gene
2001
Tyrosine nitration: Localisation, quantification, consequences for protein function and signal transduction
2001
Astrocytic production of nerve growth factor in motor neuron apoptosis: implications for amyotrophic lateral sclerosis
2004
Review
1998
BACE knockout mice are healthy despite lacking the primary beta-secretase activity in brain: implications for Alzheimer's disease therapeutics
2001
Non–cell autonomous effect of glia on motor neurons in an embryonic stem cell–based ALS model
2007
Amyotrophic lateral sclerosis
2011 Standout
Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis
2002
Evidence of widespread cerebral microglial activation in amyotrophic lateral sclerosis: an [11C](R)-PK11195 positron emission tomography study
2004
Cyclooxygenase 2 inhibition protects motor neurons and prolongs survival in a transgenic mouse model of ALS
2002
Putative function of ADAM9, ADAM10, and ADAM17 as APP -secretase
2003
Regulated Intramembrane Proteolysis
2000
Design of phase II ALS clinical trials
2008
Differential expression of inflammation‐ and apoptosis‐related genes in spinal cords of a mutant SOD1 transgenic mouse
model of familial amyotrophic lateral sclerosis
2001
Focal transplantation–based astrocyte replacement is neuroprotective in a model of motor neuron disease
2008
Increased 3‐nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis
1997
Mutant Cu/Zn-Superoxide Dismutase Proteins Have Altered Solubility and Interact with Heat Shock/Stress Proteins in Models of Amyotrophic Lateral Sclerosis
2001
Nitric Oxide and Peroxynitrite in Health and Disease
2007 Standout
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
2008
Up‐Regulation of Protein Chaperones Preserves Viability of Cells Expressing Toxic Cu/Zn‐Superoxide Dismutase Mutants Associated with Amyotrophic Lateral Sclerosis
1999
Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
2007 Standout
Decoding ALS: from genes to mechanism
2016 StandoutNature
Oxidative stress in neurodegenerative diseases: therapeutic implications for superoxide dismutase mimetics
2003
Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis
1999
Role of SOD-1 and nitric oxide/cyclic GMP cascade on neurofilament aggregation in ALS/MND
1996
Oxidative damage to mitochondrial DNA in spinal motoneurons of transgenic ALS mice
2001
Amyotrophic Lateral Sclerosis
2001
Post-translational Processing of β-Secretase (β-Amyloid-converting Enzyme) and Its Ectodomain Shedding
2001
Transition-state analogue inhibitors of γ-secretase bind directly to presenilin-1
2000
Mitochondrial Alterations in the Spinal Cord of Patients With Sporadic Amyotrophic Lateral Sclerosis
2007
Calcium dyshomeostasis and intracellular signalling in alzheimer's disease
2002
Remarkable increase in cerebrospinal fluid 3-nitrotyrosine in patients with sporadic amyotrophic lateral sclerosis
1999
TANGO1 Facilitates Cargo Loading at Endoplasmic Reticulum Exit Sites
2009 StandoutNobel
Energetics in the pathogenesis of neurodegenerative diseases
2000
Chemical remodelling of cell surfaces in living animals
2004 StandoutNatureNobel
Lack of involvement of neuronal nitric oxide synthase in the pathogenesis of a transgenic mouse model of familial amyotrophic lateral sclerosis
1999
A β-arrestin 2 Signaling Complex Mediates Lithium Action on Behavior
2008 StandoutNobel
A role for astrocytes in motor neuron loss in amyotrophic lateral sclerosis
2004
α-Synuclein Cooperates with CSPα in Preventing Neurodegeneration
2005 StandoutNobel
Mammalian APH-1 Interacts with Presenilin and Nicastrin and Is Required for Intramembrane Proteolysis of Amyloid-β Precursor Protein and Notch
2002
Minocycline Slows Disease Progression in a Mouse Model of Amyotrophic Lateral Sclerosis
2002
The Role of Presenilins in γ-Secretase Activity
2001
Truncated wild-type SOD1 and FALS-linked mutant SOD1 cause neural cell death in the chick embryo spinal cord
2005
A Switch in Retrograde Signaling from Survival to Stress in Rapid-Onset Neurodegeneration
2009
γ-Secretase Complex Assembly within the Early Secretory Pathway
2005
Requirements for Presenilin-Dependent Cleavage of Notch and Other Transmembrane Proteins
2000
Defective axonal transport in a transgenic mouse model of amyotrophic lateral sclerosis
1995 Nature
Taking dendritic cells into medicine
2007 StandoutNatureNobel
Effect of overexpression of wild‐type and mutant Cu/Zn‐superoxide dismutases on oxidative damage and antioxidant defences: relevance to Down's syndrome and familial amyotrophic lateral sclerosis
2001
Colocalization of NOS and SOD1 in neurofilament accumulation within motor neurons of amyotrophic lateral sclerosis: an immunohistochemical study
1996
The Role of Muscle microRNAs in Repairing the Neuromuscular Junction
2014
Crystal structure of an HIV-binding recombinant fragment of human CD4
1990 StandoutNatureNobel
Chapter 19 Role of endogenous nitric oxide and peroxynitrite formation in the survival and death of motor neurons in culture
1998
BACE1 is the major β-secretase for generation of Aβ peptides by neurons
2001
PEN-2 and APH-1 Coordinately Regulate Proteolytic Processing of Presenilin 1
2003
γ-Secretase-mediated proteolysis in cell-surface-receptor signalling
2002
Hassles with Taking Out the Garbage: Aggravating Aggresomes
2002
Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
2005
Phosphorylation Regulates Intracellular Trafficking of β-Secretase
2001
Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
1996
Neuroinflammation in the pathogenesis of amyotrophic lateral sclerosis
2005
Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis
2005
Reactive oxygen species and Alzheimer's disease
1997
Identification of Two Novel Loci for Dominantly Inherited Familial Amyotrophic Lateral Sclerosis
2003 Nobel
A Fresh Look at iPS Cells
2009 StandoutNobel
Progress in the pathogenesis of amyotrophic lateral sclerosis
2001
Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients
2002
Mutant SOD1 causes motor neuron disease independent of copper chaperone–mediated copper loading
2002
Benefit of vitamin E, riluzole, and gababapentin in a transgenic model of familial amyotrophic lateral sclerosis
1996
Relevance of protein nitration in brain injury: a key pathophysiological mechanism in neurodegenerative, autoimmune, or inflammatory CNS diseases and stroke
2003
Neural apoptosis
1995
Expression of human β‐secretase in the mouse brain increases the steady‐state level of β‐amyloid
2002
Slowing of axonal transport is a very early event in the toxicity of ALS–linked SOD1 mutants to motor neurons
1999
MicroRNAs as Potential Circulating Biomarkers for Amyotrophic Lateral Sclerosis
2014
Familial Alzheimer's Disease Presenilin 1 Mutations Cause Alterations in the Conformation of Presenilin and Interactions with Amyloid Precursor Protein
2005
Immature nicastrin stabilizes APH‐1 independent of PEN‐2 and presenilin: identification of nicastrin mutants that selectively interact with APH‐1
2004
ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions
1997 Standout
Single amino-acid changes in HIV envelope affect viral tropism and receptor binding
1989 StandoutNatureNobel
A Conserved GXXXG Motif in APH-1 Is Critical for Assembly and Activity of the γ-Secretase Complex
2004
The involvement of the Golgi apparatus in the pathogenesis of amyotrophic lateral sclerosis, Alzheimer's disease, and ricin intoxication
1998
Structure-based design of non-natural amino-acid inhibitors of amyloid fibril formation
2011 StandoutNatureNobel
Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2–21.3
2006
Dorfin Localizes to the Ubiquitylated Inclusions in Parkinson's Disease, Dementia with Lewy Bodies, Multiple System Atrophy, and Amyotrophic Lateral Sclerosis
2003
Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis
2004
γ-Secretase Activity Is Associated with a Conformational Change of Nicastrin
2003
Neurodegenerative diseases and oxidative stress
2004 Standout
Oxidative stress in ALS: A mechanism of neurodegeneration and a therapeutic target
2006
Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the G93A SOD1 mouse model of ALS
2006
Oxidative stress and nitration in neurodegeneration: Cause, effect, or association?
2003
Dendritic cells and the control of immunity
1998 StandoutNatureNobel
Regulation by HIV Rev depends upon recognition of splice sites
1989 StandoutNobel
Neurodegenerative diseases and oxidative stress
2003
SOD1 mutation is assosiated with accumulation of neurofilaments in amyotrophic lateral scelaries
1996
Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice
2002
Evidence That Assembly of an Active γ-Secretase Complex Occurs in the Early Compartments of the Secretory Pathway
2004
Cellular Defenses against Unfolded Proteins
2001
Novel drug development for amyotrophic lateral sclerosis
2000
Presence of dendritic cells, MCP‐1, and activated microglia/macrophages in amyotrophic lateral sclerosis spinal cord tissue
2003
Molecular biology of amyotrophic lateral sclerosis: insights from genetics
2006
Clinical neurophysiology of ALS
2001
Induction of nitrotyrosine-like immunoreactivity in the lower motor neuron of amyotrophic lateral sclerosis
1995
Gain in functions of mutant Cu,Zn-superoxide dismutases as a causative factor in familial amyotrophic lateral sclerosis: Less reactive oxidant formation but high spontaneous aggregation and precipitation
2000
Increased mitochondrial antioxidative activity or decreased oxygen free radical propagation prevent mutant SOD1‐mediated motor neuron cell death and increase amyotrophic lateral sclerosis‐like transgenic mouse survival
2002
Motoneuron Death Triggered by a Specific Pathway Downstream of Fas
2002
A Unique Microglia Type Associated with Restricting Development of Alzheimer’s Disease
2017 Standout
Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice
2004
Substrate and Inhibitor Profile of BACE (β-Secretase) and Comparison with Other Mammalian Aspartic Proteases
2002
Aggresomes, inclusion bodies and protein aggregation
2000 Standout
ALS-Linked Cu/Zn–SOD Mutation Increases Vulnerability of Motor Neurons to Excitotoxicity by a Mechanism Involving Increased Oxidative Stress and Perturbed Calcium Homeostasis
1999
Characterization of α2,6-Sialyltransferase Cleavage by Alzheimer's β-Secretase (BACE1)
2003
Amyotrophic Lateral Sclerosis
2001 Standout
Reversal of the superoxide dismutase reaction revisited
2003
Glia cells in amyotrophic lateral sclerosis: New clues to understanding an old disease?
2007
Mysteries of HIV: challenges for therapy and prevention
1988 Nature
Pathways towards and away from Alzheimer's disease
2004 StandoutNature
Partial Purification and Characterization of γ-Secretase from Post-mortem Human Brain
2003
Protein modification by the lipid peroxidation product 4‐hydroxynonenal in the spinal cords of amyotrophic lateral sclerosis patients
1998
Lost in translation: Treatment trials in the SOD1 mouse and in human ALS
2007
Peroxynitrite triggers a phenotypic transformation in spinal cord astrocytes that induces motor neuron apoptosis
2001
Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice
2002 Nature
Peroxynitrite-Induced Tyrosine Nitration and Inhibition of Protein Kinase C
2001
The Tumor Necrosis Factor Ligand and Receptor Families
1996 StandoutNobel
Dorfin Ubiquitylates Mutant SOD1 and Prevents Mutant SOD1-mediated Neurotoxicity
2002
The Deacetylase HDAC6 Regulates Aggresome Formation and Cell Viability in Response to Misfolded Protein Stress
2003 Standout
Cytoskeletal abnormalities in amyotrophic lateral sclerosis: beneficial or detrimental effects?
2000
The role of presenilin cofactors in the γ-secretase complex
2003 Nature
On the Relation of Oxidative Stress to Neuroinflammation: Lessons Learned from the G93A-SOD1 Mouse Model of Amyotrophic Lateral Sclerosis
2006
Effects of anti-gp120 monoclonal antibodies on CD4 receptor binding by the env protein of human immunodeficiency virus type 1
1988
Intermolecular transmission of superoxide dismutase 1 misfolding in living cells
2011
Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria
2006
The initial substrate-binding site of γ-secretase is located on presenilin near the active site
2005
γ-Secretase activity requires the presenilin-dependent trafficking of nicastrin through the Golgi apparatus but not its complex glycosylation
2003
High concentrations of recombinant soluble CD4 are required to neutralize primary human immunodeficiency virus type 1 isolates.
1990
Nitric Oxide and Superoxide Contribute to Motor Neuron Apoptosis Induced by Trophic Factor Deprivation
1998
Bcl-2: Prolonging Life in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis
1997 Science
Alzheimer's disease cybrids replicate beta-amyloid abnormalities through cell death pathways.
2000
CD147 is a regulatory subunit of the γ-secretase complex in Alzheimer's disease amyloid β-peptide production
2005
The Amyloid Hypothesis of Alzheimer's Disease: Progress and Problems on the Road to Therapeutics
2002 StandoutScience
Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)
2002
γ-Secretase is a membrane protein complex comprised of presenilin, nicastrin, aph-1, and pen-2
2003
Apoptosis: A Review of Programmed Cell Death
2007 Standout
PROTOFIBRILS , PORES , FIBRILS, AND NEURODEGENERATION : Separating the Responsible Protein Aggregates from The Innocent Bystanders
2003
A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide.
1996
Proteasome inhibition enhances the stability of mouse CuZn superoxide dismutase with mutations linked to familial amyotrophic lateral sclerosis
1996
In Vitro Characterization of the Presenilin-Dependent γ-Secretase Complex Using a Novel Affinity Ligand
2003
Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models
2007
Survival in transgenic ALS mice does not vary with CNS glutathione peroxidase activity
2002
Neuron-Specific Expression of Mutant Superoxide Dismutase 1 in Transgenic Mice Does Not Lead to Motor Impairment
2001
Bicarbonate enhances alpha-synuclein oligomerization and nitration: intermediacy of carbonate radical anion and nitrogen dioxide radical
2004
Functional Role of Caspase-1 and Caspase-3 in an ALS Transgenic Mouse Model
2000 Science
The Human Immunodeficiency Virus: Infectivity and Mechanisms of Pathogenesis
1988 StandoutScience
Misfolded CuZnSOD and amyotrophic lateral sclerosis
2003
High Rate of HTLV-II Infection in Seropositive IV Drug Abusers in New Orleans
1989 Science
Accumulation of SOD1 Mutants in Postnatal Motoneurons Does Not Cause Motoneuron Pathology or Motoneuron Disease
2002
Binding of F-spondin to amyloid-β precursor protein: A candidate amyloid-β precursor protein ligand that modulates amyloid-β precursor protein cleavage
2004 StandoutNobel
Oxidative stress and nitration in neurodegeneration: Cause, effect, or association?
2003
Superoxide dismutase and neurofilament transgenic models of amyotrophic lateral sclerosis
1998
In vivo genome editing improves motor function and extends survival in a mouse model of ALS
2017
Conservation of the biochemical mechanisms of signal transduction among mammalian Notch family members
2001 StandoutNobel
Nitric oxide, superoxide, and peroxynitrite: the good, the bad, and ugly
1996 Standout
Moonlighting activity of presenilin in plants is independent of γ-secretase and evolutionarily conserved
2007
Neural Science
2000 StandoutNobel
Effect of overexpression of wild‐type and mutant Cu/Zn‐superoxide dismutases on oxidative stress and cell death induced by hydrogen peroxide, 4‐hydroxynonenal or serum deprivation: potentiation of injury by ALS‐related mutant superoxide dismutases and protection by Bcl‐2
2001
Alzheimer's Disease: Genes, Proteins, and Therapy
2001 Standout
From Charcot to SOD1
1999
Superoxide dismutase and the death of motoneurons in ALS
2001
Mutations in copper-zinc superoxide dismutase that cause amyotrophic lateral sclerosis alter the zinc binding site and the redox behavior of the protein.
1996
Neurodegeneration in amyotrophic lateral sclerosis: the role of oxidative stress and altered homeostasis of metals
2003
Riluzole preserves motor function in a transgenic model of familial amyotrophic lateral sclerosis
1998
Interleukin 6: A Multifunctional Cytokine Regulating Immune Reactions and the Acute Phase Protein Response
1990
Toxic Proteins in Neurodegenerative Disease
2002 Science
Familial amyotrophic lateral sclerosis
1997
Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant
1998
Clustering of ALS patients in central Italy due to the occurrence of the L84F SOD1 gene mutation
1999
Gold-Based Therapeutic Agents
1999
Viruses in Human Cancers
1991 StandoutScienceNobel
Epitope mapping of the human immunodeficiency virus type 1 gp120 with monoclonal antibodies
1988
Behavior
2006
Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis
2006
Alzheimer's Disease: Molecular Understanding Predicts Amyloid-Based Therapeutics
2003
Immune reactivity in a mouse model of familial ALS correlates with disease progression
2001
Mitochondria and Apoptosis
1998 StandoutScience
Global Loss of Na,K-ATPase and Its Nitric Oxide-Mediated Regulation in a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis
2003
Late Onset Death of Motor Neurons in Mice Overexpressing Wild-Type Peripherin
1999
nNOS Expression in Reactive Astrocytes Correlates with Increased Cell Death Related DNA Damage in the Hippocampus and Entorhinal Cortex in Alzheimer's Disease
2000
Pathogenesis of Infection with Human Immunodeficiency Virus
1987
Biogenesis of γ-secretase early in the secretory pathway
2007 StandoutNobel
Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase
1998
Excitotoxicity and Amyotrophic Lateral Sclerosis
2005
DEVELOPMENT OF THE VERTEBRATE NEUROMUSCULAR JUNCTION
1999
Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells
2011
UNRAVELING THE MECHANISMS INVOLVED IN MOTOR NEURON DEGENERATION IN ALS
2004
Advanced Glycation Endproducts in Neurofilament Conglomeration of Motoneurons in Familial and Sporadic Amyotrophic Lateral Sclerosis
1998
A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice
2009
The Ubiquitin-Proteasome Proteolytic Pathway: Destruction for the Sake of Construction
2002 StandoutNobel
CRISPR-Cas guides the future of genetic engineering
2018 StandoutScienceNobel
Dendritic Cells Exposed to Human Immunodeficiency Virus Type-1 Transmit a Vigorous Cytopathic Infection to CD4 + T Cells
1992 StandoutScienceNobel
Restricted Expression of G86R Cu/Zn Superoxide Dismutase in Astrocytes Results in Astrocytosis But Does Not Cause Motoneuron Degeneration
2000
Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis
2000
Apoptosis in AIDS
1993 StandoutScienceNobel
Altered Reactivity of Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis
1996 Science
Synaptic Assembly of the Brain in the Absence of Neurotransmitter Secretion
2000 StandoutScienceNobel
Free Radicals in the Physiological Control of Cell Function
2002 Standout
Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase
2000
COPPER-ZINC SUPEROXIDE DISMUTASE AND AMYOTROPHIC LATERAL SCLEROSIS
2003
Elevated free nitrotyrosine levels, but not protein-bound nitrotyrosine or hydroxyl radicals, throughout amyotrophic lateral sclerosis (ALS)-like disease implicate tyrosine nitration as an aberrant in vivo property of one familial ALS-linked superoxide dismutase 1 mutant
1997
Oxidation versus aggregation — how do SOD1 mutants cause ALS?
2000
Creatine and Creatinine Metabolism
2000 Standout
Cleavage of Amyloid-β Precursor Protein and Amyloid-β Precursor-like Protein by BACE 1
2004 StandoutNobel
Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1
1998 Science
Oxidative stress in neurodegeneration: cause or consequence?
2004 Standout
Deletions of the heavy neurofilament subunit tail in amyotrophic lateral sclerosis
1999
Works of Mark E. Gurney being referenced
Presence of Cu/Zn superoxide dismutase (SOD) immunoreactivity in neuronal hyaline inclusions in spinal cords from mice carrying a transgene for Gly93Ala mutant human Cu/Zn SOD
1998
Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS)
1995
Aurothiolates Inhibit HIV-1 Infectivity by Gold(I) Ligand Exchange with a Component of the Virion Surface
1993
aph-1 and pen-2 Are Required for Notch Pathway Signaling, γ-Secretase Cleavage of βAPP, and Presenilin Protein Accumulation
2002
Enhanced oxygen radical production in a transgenic mouse model of familial amyotrophic lateral sclerosis
1998
What transgenic mice tell us about neurodegenerative disease
2000
The Copper Chelator d‐Penicillamine Delays Onset of Disease and Extends Survival in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis
1997
Systemic injections of ciliary neurotrophic factor induce sprouting by adult motor neurons>
1994
Transgenic animal models of familial amyotrophic lateral sclerosis
1997
Screening for Presenilin Inhibitors Using the Free-Living Nematode, Caenorhabditis elegans
2004
Membrane-anchored aspartyl protease with Alzheimer's disease β-secretase activity
1999 Nature
A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase ( SOD1 ) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis
1997
Relationship of oxygen radical-induced lipid peroxidative damage to disease onset and progression in a transgenic model of familial ALS
1998
Disease mechanisms revealed by transcription profiling in SOD1‐G93A transgenic mouse spinal cord
2001
Age-Dependent Penetrance of Disease in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis
1995
Intracellular Calcium Parallels Motoneuron Degeneration in SOD-1 Mutant Mice
1998
Bicarbonate Enhances Peroxidase Activity of Cu,Zn-Superoxide Dismutase
2002
Restricted heterogeneity of antibody to gp120 and p24 in AIDS.
1988
Pathogenic mechanisms in familial amyotrophic lateral sclerosis due to mutation of Cu, Zn superoxide dismutase.
1996
Molecular Cloning and Expression of Neuroleukin, a Neurotrophic Factor for Spinal and Sensory Neurons
1986 Science
Second Conserved Domain of gp120 Is Important for HIV Infectivity and Antibody Neutralization
1988 Science
Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis.
1994
Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis
2000
Immunocytochemical study on the distribution of nitrotyrosine in the brain of the transgenic mice expressing a human Cu/Zn SOD mutation
2000
Oxidative stress, mutant SOD1, and neurofilament pathology in transgenic mouse models of human motor neuron disease.
1997
Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS
1998
Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.
1996
Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation
1994 StandoutScience
The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease.
1996
Neuroleukin: A Lymphokine Product of Lectin-Stimulated T Cells
1986 Science
Brain‐derived neurotrophic factor transiently stabilizes silent synapses on developing neuromuscular junctions
1996
Reactive astrocytes express nitric oxide synthase in the spinal cord of transgenic mice expressing a human Cu/Zn SOD mutation
1998
Response : Mutant Mice, Cu,Zn Superoxide Dismutase, and Motor Neuron Degeneration
1994 Science
Functional Interaction and Partial Homology Between Human Immunodeficiency Virus and Neuroleukin
1987 Science
Relationship of oxygen radical‐induced lipid peroxidative damage to disease onset and progression in a transgenic model of familial ALS
1998