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Genetically engineered models relevant to neurodegenerative disorders: their value for understanding disease mechanisms and designing/testing experimental therapeutics

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Europe PubMed Central

PubMed publication ID

2 August 2017

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Europe PubMed Central

Genetically engineered models relevant to neurodegenerative disorders: their value for understanding disease mechanisms and designing/testing experimental therapeutics (English)

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

neurodegeneration

1 reference

based on heuristic

inferred from title

author name string

P C Wong

1

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

H Cai

2

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

D R Borchelt

3

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

D L Price

4

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

publication date

1 October 2001

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

Journal of Molecular Neuroscience

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

17

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

2

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

233-257

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

Membrane-anchored aspartyl protease with Alzheimer's disease beta-secretase activity

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Delivering copper inside yeast and human cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nicastrin modulates presenilin-mediated notch/glp-1 signal transduction and betaAPP processing

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protein topology of presenilin 1

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Metal ion chaperone function of the soluble Cu(I) receptor Atx1.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identification and characterization of a mouse homologue of the spinal muscular atrophy-determining gene, survival motor neuron

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amyotrophic lateral sclerosis. unfolding the toxicity of the misfolded

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Purification and cloning of amyloid precursor protein beta-secretase from human brain

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Superoxide-mediated cytotoxicity in superoxide dismutase-deficient fetal fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A gain of superoxide dismutase (SOD) activity obtained with CCS, the copper metallochaperone for SOD1

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Alzheimer-type neuropathology in transgenic mice overexpressing V717F β-amyloid precursor protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A novel nuclear structure containing the survival of motor neurons protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Correlative Memory Deficits, Abeta Elevation, and Amyloid Plaques in Transgenic Mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Undetectable intracellular free copper: the requirement of a copper chaperone for superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The neurobiology of childhood spinal muscular atrophy.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presenilin Proteins Undergo Heterogeneous Endoproteolysis between Thr291and Ala299and Occur as Stable N- and C-Terminal Fragments in Normal and Alzheimer Brain Tissue

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Genetic neurodegenerative diseases: the human illness and transgenic models

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Filamentous nerve cell inclusions in neurodegenerative diseases

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inhibition of ICE slows ALS in mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intracellular pathways of copper trafficking in yeast and humans

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurofilaments and orthograde transport are reduced in ventral root axons of transgenic mice that express human SOD1 with a G93A mutation

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Excitotoxicity in ALS.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Experimental models of amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuroplasticity failure in Alzheimer's disease: bridging the gap between plaques and tangles

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

SCO1 and SCO2 act as high copy suppressors of a mitochondrial copper recruitment defect in Saccharomyces cerevisiae

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A mouse model for spinal muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Motor neurone disease and animal models

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gamma -secretase inhibitors repress thymocyte development

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identification and functional expression of HAH1, a novel human gene involved in copper homeostasis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The copper chaperone CCS directly interacts with copper/zinc superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

An increased percentage of long amyloid beta protein secreted by familial amyloid beta protein precursor (beta APP717) mutants.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The Neuropathology of CAG Repeat Diseases: Review and Update of Genetic and Molecular Features

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Photoactivated gamma-secretase inhibitors directed to the active site covalently label presenilin 1

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurofilament-deficient axons and perikaryal aggregates in viable transgenic mice expressing a neurofilament-beta-galactosidase fusion protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Altered axons and axon terminals in the lateral vestibular nucleus of the rat. Possible example of axonal remodeling

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Deletion and conversion in spinal muscular atrophy patients: is there a relationship to severity?

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Translating cell biology into therapeutic advances in Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Characterization of COX17, a yeast gene involved in copper metabolism and assembly of cytochrome oxidase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuropathology of ALS: an overview

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The gene for neuronal apoptosis inhibitory protein is partially deleted in individuals with spinal muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

beta-Amyloid precursor protein-deficient mice show reactive gliosis and decreased locomotor activity

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Lack of involvement of neuronal nitric oxide synthase in the pathogenesis of a transgenic mouse model of familial amyotrophic lateral sclerosis.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Beta-amyloid precursor protein cleavage by a membrane-bound protease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Plaque-independent disruption of neural circuits in Alzheimer's disease mouse models

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ALS, SOD and peroxynitrite

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Life and death of neurons in the aging brain

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identification of a Novel Aspartic Protease (Asp 2) as β-Secretase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identification and transport of full-length amyloid precursor proteins in rat peripheral nervous system.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Functional Role of Caspase-1 and Caspase-3 in an ALS Transgenic Mouse Model

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mice with combined gene knock-outs reveal essential and partially redundant functions of amyloid precursor protein family members.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Peripherally administered antibodies against amyloid beta-peptide enter the central nervous system and reduce pathology in a mouse model of Alzheimer disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutant genes in familial Alzheimer's disease and transgenic models

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A pathogenic mutation for probable Alzheimer's disease in the APP gene at the N-terminus of beta-amyloid

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

From Charcot to SOD1: mechanisms of selective motor neuron death in ALS.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expanding our understanding of polyglutamine diseases through mouse models

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Quantitative immunocytochemical analysis of the spinal cord in G86R superoxide dismutase transgenic mice: neurochemical correlates of selective vulnerability

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Facilitation of lin-12-mediated signalling by sel-12, a Caenorhabditis elegans S182 Alzheimer's disease gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Motor neuron disease caused by mutations in superoxide dismutase 1

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

An Alzheimer's disease-linked PS1 variant rescues the developmental abnormalities of PS1-deficient embryos.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Evidence that tumor necrosis factor alpha converting enzyme is involved in regulated alpha-secretase cleavage of the Alzheimer amyloid protein precursor

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pathogenesis of two axonopathies does not require axonal neurofilaments.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuronal death in amyotrophic lateral sclerosis is apoptosis: possible contribution of a programmed cell death mechanism

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Increased central nervous system production of extracellular matrix components and development of hydrocephalus in transgenic mice overexpressing transforming growth factor-beta 1

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expression of presenilin 1 and 2 (PS1 and PS2) in human and murine tissues

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Proximal axonal enlargement in motor neuron disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Deficiency of presenilin-1 inhibits the normal cleavage of amyloid precursor protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Reduced survival motor neuron (Smn) gene dose in mice leads to motor neuron degeneration: an animal model for spinal muscular atrophy type III

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Alzheimer's disease and senile dementia: loss of neurons in the basal forebrain

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Correlation between severity and SMN protein level in spinal muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The Alzheimer's A beta peptide induces neurodegeneration and apoptotic cell death in transgenic mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Reduced fertility in female mice lacking copper-zinc superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuronal overexpression of mutant amyloid precursor protein results in prominent deposition of cerebrovascular amyloid

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Alzheimer's disease: genetic studies and transgenic models

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amyotrophic lateral sclerosis. Insights from genetics

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Genetic classification of primary neurodegenerative disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Skeletal and CNS defects in Presenilin-1-deficient mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Deposition of beta/A4 immunoreactivity and neuronal pathology in transgenic mice expressing the carboxyl-terminal fragment of the Alzheimer amyloid precursor in the brain

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The biochemical pathway of neurofibrillary degeneration in aging and Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Endoproteolysis of presenilin 1 and accumulation of processed derivatives in vivo

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuroanatomical abnormalities in behaviorally characterized APP(V717F) transgenic mice.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Isolation and characterization of APLP2 encoding a homologue of the Alzheimer's associated amyloid beta protein precursor

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nuclear targeting defect of SMN lacking the C-terminus in a mouse model of spinal muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Beta-secretase cleavage of Alzheimer's amyloid precursor protein by the transmembrane aspartic protease BACE

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Generation of APLP2 KO mice and early postnatal lethality in APLP2/APP double KO mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Excitotoxicity hypothesis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identification of a mouse brain cDNA that encodes a protein related to the Alzheimer disease-associated amyloid beta protein precursor

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presenilin 2 deficiency causes a mild pulmonary phenotype and no changes in amyloid precursor protein processing but enhances the embryonic lethal phenotype of presenilin 1 deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Two transmembrane aspartates in presenilin-1 required for presenilin endoproteolysis and gamma-secretase activity

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The genetic and molecular mechanisms of motor neuron disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Subcellular localization and axonal transport of the survival motor neuron (SMN) protein in the developing rat spinal cord

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The ATX1 gene of Saccharomyces cerevisiae encodes a small metal homeostasis factor that protects cells against reactive oxygen toxicity

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Brain transplants of cells expressing the carboxyl-terminal fragment of the Alzheimer amyloid protein precursor cause specific neuropathology in vivo

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Effects of PS1 deficiency on membrane protein trafficking in neurons

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Genetic analysis of synaptic development and plasticity: homeostatic regulation of synaptic efficacy

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Age-related CNS disorder and early death in transgenic FVB/N mice overexpressing Alzheimer amyloid precursor proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Loss of the presynaptic vesicle protein synaptophysin in hippocampus correlates with cognitive decline in Alzheimer disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Age-related learning deficits in transgenic mice expressing the 751-amino acid isoform of human beta-amyloid precursor protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Evolution of amyloid: what normal protein folding may tell us about fibrillogenesis and disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

APPSw transgenic mice develop age-related A beta deposits and neuropil abnormalities, but no neuronal loss in CA1.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Multiple protein domains contribute to the action of the copper chaperone for superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presenilin is required for activity and nuclear access of Notch in Drosophila

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Bcl-2: prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Ovarian function in superoxide dismutase 1 and 2 knockout mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presenilin 1 is required for Notch1 and DII1 expression in the paraxial mesoderm

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurofilament functions in health and disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structure of the protease domain of memapsin 2 (beta-secretase) complexed with inhibitor

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expression analysis of BACE2 in brain and peripheral tissues

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Tangles and plaques in nondemented aging and "preclinical" Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Immunization with amyloid-beta attenuates Alzheimer-disease-like pathology in the PDAPP mouse

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

BACE1 is the major beta-secretase for generation of Abeta peptides by neurons

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutation of the beta-amyloid precursor protein in familial Alzheimer's disease increases beta-protein production

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine diseases: protein cleavage and aggregation.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presenilin 1 is linked with gamma-secretase activity in the detergent solubilized state

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

BACE2, a beta -secretase homolog, cleaves at the beta site and within the amyloid-beta region of the amyloid-beta precursor protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protein oxidation and aging

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Introduction and expression of the 400 kilobase amyloid precursor protein gene in transgenic mice [corrected]

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cognitive and neurobiologic markers of early Alzheimer disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

BACE knockout mice are healthy despite lacking the primary beta-secretase activity in brain: implications for Alzheimer's disease therapeutics

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Prospects for new restorative and neuroprotective treatments in Parkinson's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Human aspartic protease memapsin 2 cleaves the beta-secretase site of beta-amyloid precursor protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Constitutive and regulated alpha-secretase cleavage of Alzheimer's amyloid precursor protein by a disintegrin metalloprotease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurogenic phenotypes and altered Notch processing in Drosophila Presenilin mutants.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Altered metabolism of familial Alzheimer's disease-linked amyloid precursor protein variants in yeast artificial chromosome transgenic mice.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amyloid, the presenilins and Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expression of APP in brains of transgenic mice containing the entire human APP gene.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Estimation of the Genetic Contribution of Presenilin-1 and -2 Mutations in a Population-Based Study of Presenile Alzheimer Disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Superoxide dismutases

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Limited corticospinal tract involvement in amyotrophic lateral sclerosis subjects with the A4V mutation in the copper/zinc superoxide dismutase gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Embryonic lethality in mice homozygous for a processing-deficient allele of Notch1.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The copper transporter CTR1 provides an essential function in mammalian embryonic development

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A beta peptide vaccination prevents memory loss in an animal model of Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cerebral amyloid induces aberrant axonal sprouting and ectopic terminal formation in amyloid precursor protein transgenic mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identification and characterization of a spinal muscular atrophy-determining gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

hCTR1: a human gene for copper uptake identified by complementation in yeast

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presenilin-1 differentially facilitates endoproteolysis of the beta-amyloid precursor protein and Notch

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurodegenerative tauopathies: human disease and transgenic mouse models

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expression of a ubiquitous, cross-reactive homologue of the mouse beta-amyloid precursor protein (APP).

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Peripheral anti-A beta antibody alters CNS and plasma A beta clearance and decreases brain A beta burden in a mouse model of Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Alzheimer's disease. A firm base for drug development.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The copper chaperone for superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutant human presenilin 1 protects presenilin 1 null mouse against embryonic lethality and elevates Abeta1-42/43 expression

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A beta peptide immunization reduces behavioural impairment and plaques in a model of Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Comparison of neurodegenerative pathology in transgenic mice overexpressing V717F beta-amyloid precursor protein and Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Familial Alzheimer's disease-linked presenilin 1 variants elevate Abeta1-42/1-40 ratio in vitro and in vivo

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The copper chelator d-penicillamine delays onset of disease and extends survival in a transgenic mouse model of familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Precursor of amyloid protein in Alzheimer disease undergoes fast anterograde axonal transport

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Transition-state analogue inhibitors of gamma-secretase bind directly to presenilin-1.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Impaired learning and LTP in mice expressing the carboxy terminus of the Alzheimer amyloid precursor protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Alzheimer amyloid protein precursor in the rat hippocampus: transport and processing through the perforant path

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Transgenic mouse brain histopathology resembles early Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

New protease inhibitors prevent gamma-secretase-mediated production of Abeta40/42 without affecting Notch cleavage

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Formation of intermediate filament protein aggregates with disparate effects in two transgenic mouse models lacking the neurofilament light subunit

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Accelerated amyloid deposition in the brains of transgenic mice coexpressing mutant presenilin 1 and amyloid precursor proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mice deficient in BACE1, the Alzheimer's beta-secretase, have normal phenotype and abolished beta-amyloid generation

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Time course of neuropathology in the spinal cord of G86R superoxide dismutase transgenic mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11816796

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1385/JMN:17:2:233

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

Dimensions Publication ID

0 references

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

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