Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
about
There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALSDefective control of pre-messenger RNA splicing in human diseasePresymptomatic activation of the PDGF-CC pathway accelerates onset of ALS neurodegeneration.A Novel Protocol for Directed Differentiation of C9orf72-Associated Human Induced Pluripotent Stem Cells Into Contractile Skeletal Myotubes.C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumAbnormal expression of homeobox genes and transthyretin in C9ORF72 expansion carriersAlternative polyadenylation of mRNA precursors.Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal DementiaSRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits.Dysregulation of RNA Binding Protein Aggregation in Neurodegenerative Disorders.Cerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers.RNAseq Analyses Identify Tumor Necrosis Factor-Mediated Inflammation as a Major Abnormality in ALS Spinal Cord.Global Profiling of the Cellular Alternative RNA Splicing Landscape during Virus-Host InteractionsThe essential and downstream common proteins of amyotrophic lateral sclerosis: A protein-protein interaction network analysis.Retrotransposon activation contributes to neurodegeneration in a Drosophila TDP-43 model of ALSTDP-43 functions within a network of hnRNP proteins to inhibit the production of a truncated human SORT1 receptor.Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic MiceHigh-throughput analyses of hnRNP H1 dissects its multi-functional aspect.Gene expression profiles and protein-protein interaction networks in amyotrophic lateral sclerosis patients with C9orf72 mutationMotor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS-FTD-linked UBQLN2 mutations.Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106.Conserved DNA methylation combined with differential frontal cortex and cerebellar expression distinguishes C9orf72-associated and sporadic ALS, and implicates SERPINA1 in disease.RNA mis-splicing in disease.ER stress and the unfolded protein response in neurodegeneration.Elimination of Toxic Microsatellite Repeat Expansion RNA by RNA-Targeting Cas9.Old versus New Mechanisms in the Pathogenesis of ALS.Repetitive element transcripts are elevated in the brain of C9orf72 ALS/FTLD patients.Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients.Modeling the C9ORF72 repeat expansion mutation using human induced pluripotent stem cells.RNA G-quadruplexes: emerging mechanisms in diseaseInsights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.ALS and FTD: an epigenetic perspective.Molecular Genetics of Neurodegenerative Dementias.Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways.Faulty RNA splicing: consequences and therapeutic opportunities in brain and muscle disorders.Cleavage and polyadenylation: Ending the message expands gene regulation.The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains.Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia.RNA Misprocessing in C9orf72-Linked Neurodegeneration.
P2860
Q26750577-95F505C5-B7A4-4B2D-84E8-7AF8425D485EQ28077564-05E41382-BB09-4BC1-A708-CB310D68583AQ30746065-E1EE6173-17B7-4877-8BE7-A560FFD71E12Q30824665-425BF82A-EE71-43EB-88B7-06A4DE40AA02Q33614811-E31B1FB2-0CCC-439B-93F4-0CD3F3AA1143Q33653996-5E00AC02-E443-4076-AD11-095B971CAD83Q33821516-45F9EADC-51CE-4369-87C3-A08DD20CB921Q33832891-6E78B41D-01E8-4243-A1DA-6D2473C5B9A0Q33875570-A7464133-B682-47E7-A827-57AD8A0C64EAQ33891056-938D5B17-751E-4449-872A-3213CCAAE917Q34555364-7DA142A1-BB2D-418C-BF91-DA28C2381415Q36073660-D3A6D17F-E5DF-4B17-B918-8A24F1FA6DCBQ36093828-2AA123E7-B1E9-4708-8FDB-6AE611D423C4Q36124078-AABA21C7-E65B-43DC-9246-555723400726Q36304197-7D4F872A-BA41-486E-A406-1E1B5279DFF5Q36310409-7668004C-F465-4E46-8AEC-C0438077D49EQ36514627-739BC339-998F-47DF-9F29-56CD97639C97Q36788842-451FAF96-73D4-4944-A8B0-32C9CAC2926EQ36827111-1298969C-CAF6-42E8-9B61-611740BF43E9Q37396365-0DDE00FE-1B01-44A3-8E37-1F812F6DB385Q37451158-D2520CED-4105-4A57-8361-5D1637124846Q37616732-D9021AEE-52F2-4EF9-9C4B-FB1D511DD93AQ38624666-D0625686-6F4B-4AED-8DDC-E4C14474B103Q38642996-04A61763-9DA1-4CD4-BAFA-322E6658F989Q38653861-1F7A3A2B-EF1D-426D-AAB7-A33CD5BD4D6DQ38695021-ABD68E4B-EAB7-414F-A3BA-E409A066D274Q38700722-5A7F5D81-FEE6-43FF-8D7A-86CD747227F8Q38715333-6A5D44FA-3C7E-4276-B476-42BE9BF0CBC2Q38725663-11FDB709-F02D-4ECE-A9CA-A67CE75B7F7FQ38741408-A9B81D14-77A0-4CB0-BFF0-2FBF850D47BDQ38778353-DE516E0B-F354-41DC-B15A-42BC8B954EF9Q38789426-2D8A8DDF-BAE9-4466-B27F-6A969AABE8B3Q38860456-4E2E9E0B-CD98-4072-B399-CC3BDBBE4FCEQ39035458-A6D9BD30-FCC3-4D7C-9EF4-F509A7C7B155Q39103483-7ED356AF-88DC-4497-BF28-C6BB3A5F9F2AQ39256785-560862A6-7212-4479-917B-64291485E90DQ39269835-C8049464-C86B-469C-A6A3-E12E415AF89AQ39396328-93A9158F-E4C1-4907-9795-C1056E2BA5E4Q39454075-71DE87ED-C826-4F7E-A4B2-EC8773313185Q39458329-3B611CAE-3DB3-485B-9F97-0F9FB6E45DBB
P2860
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
description
2015 nî lūn-bûn
@nan
2015年の論文
@ja
2015年論文
@yue
2015年論文
@zh-hant
2015年論文
@zh-hk
2015年論文
@zh-mo
2015年論文
@zh-tw
2015年论文
@wuu
2015年论文
@zh
2015年论文
@zh-cn
name
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
@ast
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
@en
type
label
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
@ast
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
@en
prefLabel
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
@ast
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
@en
P2093
P2860
P50
P356
P1433
P1476
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.
@en
P2093
Amelia E Piazza-Johnston
Caroline T Stetler
Christian A Ross
Christopher D Link
Karen K Overstreet
Kevin B Boylan
Luc J Pregent
Mary D Davis
Matthew C Baker
P2860
P2888
P304
P356
10.1038/NN.4065
P407
P50
P577
2015-07-20T00:00:00Z
P5875
P6179
1021581975