Pathogenesis of FUS-associated ALS and FTD: insights from rodent models (Q28067147)

amyotrophic lateral sclerosis

PubMed

main subject

5 references

25 April 2019

2 August 2019

28 November 2019

18 December 2019

20 December 2019

frontotemporal dementia

5 references

25 April 2019

2 August 2019

28 November 2019

18 December 2019

20 December 2019

proto-oncogene protein

1 reference

2 August 2019

1 reference

28 November 2019

1 reference

18 December 2019

1 reference

20 December 2019

1 reference

1 reference

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31 May 2020

1 reference

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31 May 2020

Olaf Ansorge

2 references

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Acta Neuropathologica Communications

31 May 2020

language of work or name

1 reference

6 September 2016

6 references

25 April 2019

2 August 2019

28 November 2019

18 December 2019

20 December 2019

6 references

25 April 2019

2 August 2019

28 November 2019

18 December 2019

20 December 2019

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27600654%20AND%20SRC:MED&resulttype=core&format=json

31 May 2020

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27600654%20AND%20SRC:MED&resulttype=core&format=json

31 May 2020

2 references

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31 May 2020

2 references

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Creative Commons Attribution 4.0 International

31 May 2020

5 references

25 April 2019

2 August 2019

28 November 2019

18 December 2019

on focus list of Wikimedia project

20 December 2019

copyrighted

0 references

ScienceSource

0 references

cites work

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

20 March 2017

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

20 March 2017

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FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis

20 March 2017

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Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia

20 March 2017

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

20 March 2017

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A new subtype of frontotemporal lobar degeneration with FUS pathology

20 March 2017

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Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease

20 March 2017

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

20 March 2017

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Detection of arginine dimethylated peptides by parallel precursor ion scanning mass spectrometry in positive ion mode

20 March 2017

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Transportin1: a marker of FTLD-FUS

20 March 2017

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

20 March 2017

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FUS pathology in basophilic inclusion body disease

20 March 2017

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

20 March 2017

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

20 March 2017

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TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

20 March 2017

1 reference

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TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

20 March 2017

1 reference

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Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria

7 April 2017

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Protein arginine methylation in mammals: who, what, and why

7 April 2017

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De Novo and Rare Variants at Multiple Loci Support the Oligogenic Origins of Atrioventricular Septal Heart Defects.

7 April 2017

1 reference

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Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss

29 September 2017

1 reference

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Monomethylated and unmethylated FUS exhibit increased binding to Transportin and distinguish FTLD-FUS from ALS-FUS.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

29 September 2017

1 reference

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Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice

29 September 2017

1 reference

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

29 September 2017

1 reference

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Phosphorylation of C-terminal tyrosine residue 526 in FUS impairs its nuclear import.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

ALS mutations in TLS/FUS disrupt target gene expression

29 September 2017

1 reference

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FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis

29 September 2017

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Amyotrophic lateral sclerosis onset is influenced by the burden of rare variants in known amyotrophic lateral sclerosis genes.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Activity-dependent FUS dysregulation disrupts synaptic homeostasis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Frontotemporal dementia and its subtypes: a genome-wide association study

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

FUS is phosphorylated by DNA-PK and accumulates in the cytoplasm after DNA damage

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The role of FUS gene variants in neurodegenerative diseases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The epidemiology of ALS: a conspiracy of genes, environment and time

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

25 years of neuroimaging in amyotrophic lateral sclerosis.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The RNA-binding protein fused in sarcoma (FUS) functions downstream of poly(ADP-ribose) polymerase (PARP) in response to DNA damage

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The genetics and neuropathology of amyotrophic lateral sclerosis

29 September 2017

1 reference

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Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

TLS/FUS (translocated in liposarcoma/fused in sarcoma) regulates target gene transcription via single-stranded DNA response elements

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

FUS and TDP43 genetic variability in FTD and CBS

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

A yeast model of FUS/TLS-dependent cytotoxicity

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene

29 September 2017

1 reference

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Distinct pathological subtypes of FTLD-FUS.

29 September 2017

1 reference

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Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The epidemiology and treatment of ALS: focus on the heterogeneity of the disease and critical appraisal of therapeutic trials

29 September 2017

1 reference

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Oligogenic basis of isolated gonadotropin-releasing hormone deficiency.

29 September 2017

1 reference

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

29 September 2017

1 reference

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

29 September 2017

1 reference

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Fus gene mutations in familial and sporadic amyotrophic lateral sclerosis

29 September 2017

1 reference

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Functional analyses of variants reveal a significant role for dominant negative and common alleles in oligogenic Bardet-Biedl syndrome

29 September 2017

1 reference

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Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Analysis of FUS gene mutation in familial amyotrophic lateral sclerosis within an Italian cohort

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

TDP-43 A315T mutation in familial motor neuron disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Cognitive impairment in amyotrophic lateral sclerosis

29 September 2017

1 reference

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Recent advances in the genetics of amyotrophic lateral sclerosis and frontotemporal dementia: common pathways in neurodegenerative disease.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

The effects of executive and behavioral dysfunction on the course of ALS.

29 September 2017

1 reference

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Serum and cerebrospinal fluid biochemical markers of ALS.

29 September 2017

1 reference

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Clinical trials in ALS: an overview.

29 September 2017

1 reference

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

ALS-FUS pathology revisited: singleton FUS mutations and an unusual case with both a FUS and TARDBP mutation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons.

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Familial ALS with FUS P525L mutation: two Japanese sisters with multiple systems involvement.

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Evidence for an oligogenic basis of amyotrophic lateral sclerosis

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43)

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5011941

Oligogenic combinations associated with breast cancer risk in women under 53 years of age.

2 June 2018

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21 January 2018

Frontotemporal dementia.

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Oligogenic inheritance of optineurin (OPTN) and C9ORF72 mutations in ALS highlights localisation of OPTN in the TDP-43-negative inclusions of C9ORF72-ALS.

21 January 2018

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TLS and PRMT1 synergistically coactivate transcription at the survivin promoter through TLS arginine methylation

21 January 2018

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SnapShot: Genetics of ALS and FTD.

21 January 2018

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Assessment of cognitive impairment in amyotrophic lateral sclerosis

21 January 2018

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Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutation

21 January 2018

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Genetic contribution of FUS to frontotemporal lobar degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2FS40478-016-0358-8

Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: a comparative clinicopathological study

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2FS40478-016-0358-8

Autosomal dominant inheritance of rapidly progressive amyotrophic lateral sclerosis due to a truncation mutation in the fused in sarcoma (FUS) gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2FS40478-016-0358-8

Confounding effects of mimicking disorders in the early diagnosis of amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2FS40478-016-0358-8

VAPB and C9orf72 mutations in 1 familial amyotrophic lateral sclerosis patient

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2FS40478-016-0358-8

Mutations of FUS gene in sporadic amyotrophic lateral sclerosis

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/27600654

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1186/S40478-016-0358-8

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27600654%20AND%20SRC:MED&resulttype=core&format=json

Dimensions Publication ID

31 May 2020

1039143056

0 references

1 reference

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27600654%20AND%20SRC:MED&resulttype=core&format=json

31 May 2020

PubMed publication ID

27600654

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27600654%20AND%20SRC:MED&resulttype=core&format=json