(Q38086771)

28 August 2017

title

The changing scene of amyotrophic lateral sclerosis (English)

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amyotrophic lateral sclerosis

28 August 2017

main subject

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1

Wim Robberecht

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28 August 2017

Thomas Philips

2

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28 August 2017

language of work or name

English

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publication date

6 March 2013

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Nature Reviews Neuroscience

28 August 2017

published in

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28 August 2017

volume

14

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28 August 2017

issue

4

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28 August 2017

page(s)

248-264

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Selective neuronal vulnerability in neurodegenerative diseases: from stressor thresholds to degeneration

28 August 2017

cites work

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Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases

31 March 2020

1 reference

Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man

31 March 2020

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Prevalence and patterns of cognitive impairment in sporadic ALS.

31 March 2020

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Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

31 March 2020

1 reference

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

31 March 2020

1 reference

Altered sensorimotor development in a transgenic mouse model of amyotrophic lateral sclerosis

31 March 2020

1 reference

Neonatal neuronal circuitry shows hyperexcitable disturbance in a mouse model of the adult-onset neurodegenerative disease amyotrophic lateral sclerosis

31 March 2020

1 reference

Early electrophysiological abnormalities in lumbar motoneurons in a transgenic mouse model of amyotrophic lateral sclerosis

31 March 2020

1 reference

The G93C Mutation in Superoxide Dismutase 1

31 March 2020

1 reference

Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease

31 March 2020

1 reference

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

31 March 2020

1 reference

Dysfunction of constitutive and inducible ubiquitin-proteasome system in amyotrophic lateral sclerosis: implication for protein aggregation and immune response.

31 March 2020

1 reference

Insoluble Mutant SOD1 Is Partly Oligoubiquitinated in Amyotrophic Lateral Sclerosis Mice

31 March 2020

1 reference

Autophagy dysregulation in amyotrophic lateral sclerosis.

31 March 2020

1 reference

Autophagy in Spinal Cord Motor Neurons in Sporadic Amyotrophic Lateral Sclerosis

31 March 2020

1 reference

Increased autophagy in transgenic mice with a G93A mutant SOD1 gene

31 March 2020

1 reference

A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice

31 March 2020

1 reference

Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

31 March 2020

1 reference

Wild-type superoxide dismutase acquires binding and toxic properties of ALS-linked mutant forms through oxidation

31 March 2020

1 reference

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

31 March 2020

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Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis

31 March 2020

1 reference

Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS

31 March 2020

1 reference

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

31 March 2020

1 reference

Family-based association between Alzheimer's disease and variants in UBQLN1

31 March 2020

1 reference

Ubiquilin interacts with ubiquitylated proteins and proteasome through its ubiquitin-associated and ubiquitin-like domains

31 March 2020

1 reference

UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis

31 March 2020

1 reference

Screening in ALS and FTD patients reveals 3 novel UBQLN2 mutations outside the PXX domain and a pure FTD phenotype

31 March 2020

1 reference

SQSTM1 mutations in familial and sporadic amyotrophic lateral sclerosis

31 March 2020

1 reference

Exome sequencing reveals VCP mutations as a cause of familial ALS

31 March 2020

1 reference

Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

31 March 2020

1 reference

Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system

31 March 2020

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ATPase activity of p97-valosin-containing protein (VCP). D2 mediates the major enzyme activity, and D1 contributes to the heat-induced activity

31 March 2020

1 reference

ALS phenotypes with mutations in CHMP2B (charged multivesicular body protein 2B)

31 March 2020

1 reference

Mutations of optineurin in amyotrophic lateral sclerosis

31 March 2020

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Deleterious variants of FIG4, a phosphoinositide phosphatase, in patients with ALS.

31 March 2020

1 reference

Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease

31 March 2020

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Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia

31 March 2020

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Optineurin negatively regulates TNFalpha- induced NF-kappaB activation by competing with NEMO for ubiquitinated RIP

31 March 2020

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Differential involvement of optineurin in amyotrophic lateral sclerosis with or without SOD1 mutations

31 March 2020

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Regulation of Fab1 phosphatidylinositol 3-phosphate 5-kinase pathway by Vac7 protein and Fig4, a polyphosphoinositide phosphatase family member.

31 March 2020

1 reference

Selective autophagy: ubiquitin-mediated recognition and beyond

31 March 2020

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Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis

31 March 2020

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Therapy development in spinal muscular atrophy

31 March 2020

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Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?

31 March 2020

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

31 March 2020

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Valosin-containing protein and neurofibromin interact to regulate dendritic spine density

31 March 2020

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RNA metabolism and the pathogenesis of motor neuron diseases

31 March 2020

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TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

31 March 2020

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Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis

31 March 2020

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Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration

31 March 2020

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Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping

31 March 2020

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Nuclear factor TDP-43 binds to the polymorphic TG repeats in CFTR intron 8 and causes skipping of exon 9: a functional link with disease penetrance

31 March 2020

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

31 March 2020

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Characterizing the RNA targets and position-dependent splicing regulation by TDP-43.

31 March 2020

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TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes

31 March 2020

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Nuclear factor TDP-43 can affect selected microRNA levels

31 March 2020

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TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor

31 March 2020

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TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1

31 March 2020

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The Mammalian Disaggregase Machinery: Hsp110 Synergizes with Hsp70 and Hsp40 to Catalyze Protein Disaggregation and Reactivation in a Cell-Free System

31 March 2020

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TDP-43 aggregation in neurodegeneration: are stress granules the key?

31 March 2020

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Dynamic shuttling of TIA-1 accompanies the recruitment of mRNA to mammalian stress granules

31 March 2020

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RNA-binding proteins TIA-1 and TIAR link the phosphorylation of eIF-2 alpha to the assembly of mammalian stress granules

31 March 2020

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Mammalian stress granules represent sites of accumulation of stalled translation initiation complexes

31 March 2020

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Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue

31 March 2020

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TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity

31 March 2020

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ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.

31 March 2020

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Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

31 March 2020

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TDP-43-mediated neuron loss in vivo requires RNA-binding activity

31 March 2020

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TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.

31 March 2020

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TDP-43 regulates its mRNA levels through a negative feedback loop

31 March 2020

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Alpha-synuclein locus duplication as a cause of familial Parkinson's disease

31 March 2020

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APP locus duplication causes autosomal dominant early-onset Alzheimer disease with cerebral amyloid angiopathy

31 March 2020

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Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways

31 March 2020

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Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT.

31 March 2020

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Expanded ATXN2 CAG repeat size in ALS identifies genetic overlap between ALS and SCA2.

31 March 2020

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Ataxin-1 and ataxin-2 intermediate-length PolyQ expansions in amyotrophic lateral sclerosis

31 March 2020

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Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients

31 March 2020

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The modulation of Amyotrophic Lateral Sclerosis risk by ataxin-2 intermediate polyglutamine expansions is a specific effect

31 March 2020

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PolyQ repeat expansions in ATXN2 associated with ALS are CAA interrupted repeats

31 March 2020

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ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications

31 March 2020

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Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models

31 March 2020

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Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis

31 March 2020

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Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS

31 March 2020

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TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

31 March 2020

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Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6

31 March 2020

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Novel missense and truncating mutations in FUS/TLS in familial ALS

31 March 2020

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Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions

31 March 2020

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Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations

31 March 2020

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Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain

31 March 2020

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

31 March 2020

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Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS.

31 March 2020

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

31 March 2020

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TDP-43 and FUS: a nuclear affair

31 March 2020

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Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis

31 March 2020

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A yeast functional screen predicts new candidate ALS disease genes

31 March 2020

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Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis

31 March 2020

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The seeds of neurodegeneration: prion-like spreading in ALS

31 March 2020

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The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease.

31 March 2020

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Prion-like disorders: blurring the divide between transmissibility and infectivity

31 March 2020

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Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells

31 March 2020

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Intermolecular transmission of superoxide dismutase 1 misfolding in living cells

31 March 2020

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ALS motor phenotype heterogeneity, focality, and spread: deconstructing motor neuron degeneration

31 March 2020

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Pathological α-synuclein transmission initiates Parkinson-like neurodegeneration in nontransgenic mice

31 March 2020

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Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

31 March 2020

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A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

31 March 2020

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A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study

31 March 2020

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Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study

31 March 2020

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C9ORF72 repeat expansion in clinical and neuropathologic frontotemporal dementia cohorts.

31 March 2020

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The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs

31 March 2020

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Clinical characteristics of patients with familial amyotrophic lateral sclerosis carrying the pathogenic GGGGCC hexanucleotide repeat expansion of C9ORF72.

31 March 2020

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Phenotype difference between ALS patients with expanded repeats in C9ORF72 and patients with mutations in other ALS-related genes

31 March 2020

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Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion

31 March 2020

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Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study

31 March 2020

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Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations

31 March 2020

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Familial frontotemporal dementia with amyotrophic lateral sclerosis and a shared haplotype on chromosome 9p

31 March 2020

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Evidence for an oligogenic basis of amyotrophic lateral sclerosis

31 March 2020

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How do C9ORF72 repeat expansions cause amyotrophic lateral sclerosis and frontotemporal dementia: can we learn from other noncoding repeat expansion disorders?

31 March 2020

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Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p

31 March 2020

1 reference

Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

31 March 2020

1 reference

Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21

31 March 2020

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Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17

31 March 2020

1 reference

Myotonic dystrophy mutation: an unstable CTG repeat in the 3' untranslated region of the gene

31 March 2020

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Myotonic dystrophy type 2 caused by a CCTG expansion in intron 1 of ZNF9

31 March 2020

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Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy

31 March 2020

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Disruption of Splicing Regulated by a CUG-Binding Protein in Myotonic Dystrophy

31 March 2020

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Dysregulation of human brain microtubule-associated tau mRNA maturation in myotonic dystrophy type 1.

31 March 2020

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Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy

31 March 2020

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Muscleblind-like 1 knockout mice reveal novel splicing defects in the myotonic dystrophy brain

31 March 2020

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A muscleblind knockout model for myotonic dystrophy

31 March 2020

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C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexes

31 March 2020

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Repeat Associated Non-ATG Translation Initiation: One DNA, Two Transcripts, Seven Reading Frames, Potentially Nine Toxic Entities!

31 March 2020

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Non-ATG-initiated translation directed by microsatellite expansions

31 March 2020

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The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.

31 March 2020

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Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS

31 March 2020

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Widespread multiple system degeneration in a patient with familial amyotrophic lateral sclerosis

31 March 2020

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Motor neuron diversity in development and disease

31 March 2020

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Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF

31 March 2020

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EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans

31 March 2020

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Glutamate excitotoxicity and therapeutic targets for amyotrophic lateral sclerosis.

31 March 2020

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Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis

31 March 2020

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

31 March 2020

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Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase

31 March 2020

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Glial D-serine gates NMDA receptors at excitatory synapses in prefrontal cortex

31 March 2020

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Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons

31 March 2020

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A single amino acid residue determines the Ca2+ permeability of AMPA-selective glutamate receptor channels

31 March 2020

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Astrocytes regulate GluR2 expression in motor neurons and their vulnerability to excitotoxicity

31 March 2020

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Calcium dynamics and buffering in oculomotor neurones from mouse that are particularly resistant during amyotrophic lateral sclerosis (ALS)-related motoneurone disease

31 March 2020

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Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

31 March 2020

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ALS: a disease of motor neurons and their nonneuronal neighbors

31 March 2020

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Onset and progression in inherited ALS determined by motor neurons and microglia

31 March 2020

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Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice.

31 March 2020

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The effect of mutant SOD1 dismutase activity on non-cell autonomous degeneration in familial amyotrophic lateral sclerosis

31 March 2020

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Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

31 March 2020

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Modulating inflammatory monocytes with a unique microRNA gene signature ameliorates murine ALS.

31 March 2020

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CD4+ T cells support glial neuroprotection, slow disease progression, and modify glial morphology in an animal model of inherited ALS.

31 March 2020

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T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS.

31 March 2020

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Lymphocytic infiltrates in the spinal cord in amyotrophic lateral sclerosis

31 March 2020

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Endogenous regulatory T lymphocytes ameliorate amyotrophic lateral sclerosis in mice and correlate with disease progression in patients with amyotrophic lateral sclerosis

31 March 2020

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Regulatory T-lymphocytes mediate amyotrophic lateral sclerosis progression and survival

31 March 2020

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Oligodendroglia metabolically support axons and contribute to neurodegeneration.

31 March 2020

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Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis

31 March 2020

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Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis

31 March 2020

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MicroRNA-206 delays ALS progression and promotes regeneration of neuromuscular synapses in mice

31 March 2020

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ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration

31 March 2020

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Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice.

31 March 2020

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Axon guidance proteins: novel therapeutic targets for ALS?

31 March 2020

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Neurofilament light and polyadenylated mRNA levels are decreased in amyotrophic lateral sclerosis motor neurons

31 March 2020

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TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein

31 March 2020

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Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS).

31 March 2020

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A frameshift deletion in peripherin gene associated with amyotrophic lateral sclerosis

31 March 2020

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Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis

31 March 2020

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Novel insertion in the KSP region of the neurofilament heavy gene in amyotrophic lateral sclerosis (ALS)

31 March 2020

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Mutant dynactin in motor neuron disease.

31 March 2020

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Point mutations of the p150 subunit of dynactin (DCTN1) gene in ALS.

31 March 2020

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Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

31 March 2020

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EphA4 catalytic activity causes inhibition of RhoA GTPase in Xenopus laevis embryos

31 March 2020

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The amyotrophic lateral sclerosis 8 protein VAPB is cleaved, secreted, and acts as a ligand for Eph receptors

31 March 2020

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A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis

31 March 2020

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The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis

31 March 2020

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Diverse roles of Rho family GTPases in neuronal development, survival, and death

31 March 2020

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Nogo provides a molecular marker for diagnosis of amyotrophic lateral sclerosis

31 March 2020

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The neurite outgrowth inhibitor Nogo-A promotes denervation in an amyotrophic lateral sclerosis model

31 March 2020

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VEGF mediates commissural axon chemoattraction through its receptor Flk1.

31 March 2020

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Abnormal blood vessel development and lethality in embryos lacking a single VEGF allele

31 March 2020

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Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS.

31 March 2020

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Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis

31 March 2020

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Kinesin-associated protein 3 (KIFAP3) has no effect on survival in a population-based cohort of ALS patients

31 March 2020

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Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis

31 March 2020

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UNC13A is a modifier of survival in amyotrophic lateral sclerosis

31 March 2020

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Variants of the elongator protein 3 (ELP3) gene are associated with motor neuron degeneration

31 March 2020

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DNA repair defects sensitize cells to anticodon nuclease yeast killer toxins.

31 March 2020

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ELP3 controls active zone morphology by acetylating the ELKS family member Bruchpilot

31 March 2020

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TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

31 March 2020

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Frontotemporal dementia and motor neurone disease: overlapping clinic-pathological disorders

31 March 2020

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Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations.

31 March 2020

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Motor neuron dysfunction in frontotemporal dementia.

31 March 2020

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The overlap of amyotrophic lateral sclerosis and frontotemporal dementia

31 March 2020

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Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS.

31 March 2020

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Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior

31 March 2020

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Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy

31 March 2020

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Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis

31 March 2020

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Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice

31 March 2020

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Phosphorylation Promotes Neurotoxicity in aCaenorhabditis elegansModel of TDP-43 Proteinopathy

31 March 2020

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Understanding the role of TDP-43 and FUS/TLS in ALS and beyond.

31 March 2020

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Progressive motor weakness in transgenic mice expressing human TDP-43.

31 March 2020

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TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

31 March 2020

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TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration

31 March 2020

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Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.

31 March 2020

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Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43

31 March 2020

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Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats

31 March 2020

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Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death.

31 March 2020

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Male sterility and enhanced radiation sensitivity in TLS(-/-) mice

31 March 2020

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The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology.

31 March 2020

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FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration

31 March 2020

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Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion

31 March 2020

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Antisense oligonucleotide therapy for neurodegenerative disease.

31 March 2020

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Targeting nuclear RNA for in vivo correction of myotonic dystrophy

31 March 2020

1 reference

Fragment C of tetanus toxin, more than a carrier. Novel perspectives in non-viral ALS gene therapy

31 March 2020

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Intravascular AAV9 preferentially targets neonatal neurons and adult astrocytes

31 March 2020

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Stem Cell Transplantation for Motor Neuron Disease: Current Approaches and Future Perspectives

31 March 2020

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Induced pluripotent stem cells generated from patients with ALS can be differentiated into motor neurons

31 March 2020

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Lumbar intraspinal injection of neural stem cells in patients with amyotrophic lateral sclerosis: results of a phase I trial in 12 patients.

31 March 2020

1 reference

Focal transplantation-based astrocyte replacement is neuroprotective in a model of motor neuron disease

31 March 2020

1 reference

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

31 March 2020

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ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis

31 March 2020

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DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4)

31 March 2020

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SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis

31 March 2020

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A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2

31 March 2020

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A novel locus for familial amyotrophic lateral sclerosis, on chromosome 18q

31 March 2020

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Identification of two novel loci for dominantly inherited familial amyotrophic lateral sclerosis

31 March 2020

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Linkage of a commoner form of recessive amyotrophic lateral sclerosis to chromosome 15q15-q22 markers

31 March 2020

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Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells

31 March 2020

1 reference

12 December 2020

TLS (FUS) binds RNA in vivo and engages in nucleo-cytoplasmic shuttling

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12 December 2020

The microglial-motoneuron dialogue in ALS

1 reference

12 December 2020

Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3' end of a transcript encoding a protein kinase family member

1 reference

12 December 2020

Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS

1 reference

12 December 2020

hTAF(II)68, a novel RNA/ssDNA-binding protein with homology to the pro-oncoproteins TLS/FUS and EWS is associated with both TFIID and RNA polymerase II

1 reference

12 December 2020

Identifiers

DOI

10.1038/NRN3430

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Dimensions Publication ID

28 August 2017

1025757864

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