Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS. - Wikidata - awgldk - TriplyDB

Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.

Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.

http://www.wikidata.org/entity/Q42693496

about

There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS Modeling diseases of noncoding unstable repeat expansions using mutant pluripotent stem cells.TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Drosophila as an In Vivo Model for Human Neurodegenerative Disease Intrinsic disorder in proteins involved in amyotrophic lateral sclerosis.Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD.Dipeptide repeat protein inclusions are rare in the spinal cord and almost absent from motor neurons in C9ORF72 mutant amyotrophic lateral sclerosis and are unlikely to cause their degeneration.FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions Cerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers.Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity.C9orf72 mutations do not influence the tau signature of amyotrophic lateral sclerosis with cognitive impairment (ALSci).Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins.The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the Proteasome The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.RAN translation-What makes it run?Genetics of Frontotemporal Lobar Degeneration: From the Bench to the Clinic.Inhibition of retrograde transport modulates misfolded protein accumulation and clearance in motoneuron diseases.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.Decoding ALS: from genes to mechanism.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.CGG Repeat-Associated Non-AUG Translation Utilizes a Cap-Dependent Scanning Mechanism of Initiation to Produce Toxic Proteins.Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.ALS-FUS pathology revisited: singleton FUS mutations and an unusual case with both a FUS and TARDBP mutation Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS.Regulation of mRNA Translation in Neurons-A Matter of Life and Death.Repeat-associated non-AUG translation from antisense CCG repeats in fragile X tremor/ataxia syndrome.The Genetics of Monogenic Frontotemporal Dementia.A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism.RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response.RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration.Non-AUG translation: a new start for protein synthesis in eukaryotes.C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.In Situ Structure of Neuronal C9orf72 Poly-GA Aggregates Reveals Proteasome Recruitment.C-terminal short arginine/serine repeat sequence-dependent regulation of Y14 (RBM8A) localization.Cerebellar neuronal loss in amyotrophic lateral sclerosis cases with ATXN2 intermediate repeat expansions.

P2860

Q26750577-A22FEAEE-5CE5-4FBE-BE71-CC636DD1446C Q27687330-DBDFE6CD-8B42-4091-824A-652091E36E83 Q28080628-EAD21194-97B3-42C3-B913-1793A874EE26 Q28088777-D85B2D40-2172-4516-BF87-FCF128EE6823 Q30395169-1B6EDB7A-B8F2-4751-902C-E67232A9376C Q33577239-0090B031-6A85-4648-A092-D1E179117CED Q33614771-C0D153B8-BC21-426C-BBE3-E9BE3C430110 Q35776562-7B3AE4FB-EB4E-4FF0-956D-23C5DFBE945D Q36073654-957924B9-1816-490E-AA4A-48D3AA07D617 Q36073660-3D7A6250-8C03-4504-9825-C2E83BE70CF2 Q36073675-AC5810C9-2C00-4C24-A1D9-EB9A46D22C38 Q36173153-6B8B3D81-F0EA-44A2-ABCE-660FB08D4C3C Q36386157-53D1D642-598E-4EDB-B3AD-BC999D4F47F2 Q36795394-8273923C-88F3-4825-B8A4-0D04FA432648 Q37178589-1D0C7539-1991-4D7C-8C71-4AD4A2AF41BF Q37476206-A295D7EE-62D8-4693-8F1E-5B927FC1314A Q37615294-8DC8879C-5937-49C4-90F6-4E5286A6B174 Q38728380-081966B8-AF62-4E66-9C90-00EE139CC08C Q38789426-58EC83ED-5F1B-4FEB-B09F-8E00504F3575 Q38802827-325504EE-37CF-4A2A-9D8D-513FE2EDA3FD Q38814847-DB3AA025-D364-4F83-9261-4AF2692DACC8 Q38840491-E29C66E0-B3BE-4799-A195-42CE1CC09A05 Q38952327-30F9A61C-E2BC-4296-8F4A-9085757CC86B Q39004348-FE594D4D-1533-4B07-8FDB-8BA2ECC4C0FC Q39153670-8DC9094A-D150-49E1-A750-097003BC99AB Q39866921-CAB96F7D-4310-492A-8E54-BA87FA03D856 Q40287573-5AA63F5F-9D83-44DA-9E22-2353D307401F Q42003982-60AB8EE6-E26A-47BE-93B3-A943B206DA0B Q42925023-32867714-4CEE-4B17-BE2B-EE8996EDE595 Q46818543-03C27E7B-1B5E-4117-83F6-C8059A83E9E2 Q46839217-097B6CB2-66EA-4EDD-A725-B2A444A81483 Q46958756-5E8C3982-46A9-403F-A45B-2D3B75FEC2EE Q47230701-CBFCCEBD-63CF-47B9-9E4D-BD009D63AD9F Q47313248-3B0172A1-A2C0-42BF-909B-250268C7A60F Q47423589-9CA20170-F5A4-4466-84C9-68CAF6B2664C Q47690525-F9561462-45E4-4FE3-AA9A-8FA3EEA8D036 Q47707045-CC293F3C-A48A-4CBE-B168-D3909011666C Q49344570-C534DE83-A97E-42CD-B886-075FC49C91F8 Q49445321-E26F4CD2-9354-4FEF-B726-64B1AD93D0CC Q52146520-E11D8868-85B6-47B9-A4F5-240FE04FAC42

P2860

Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.

http://www.wikidata.org/entity/Q42693496

description

2014 nî lūn-bûn

@nan

2014年の論文

@ja

2014年論文

@yue

2014年論文

@zh-hant

2014年論文

@zh-hk

2014年論文

@zh-mo

2014年論文

@zh-tw

2014年论文

@wuu

2014年论文

@zh

2014年论文

@zh-cn

name

Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.

@en

Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.

@nl

type

label

Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.

@en

Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.

@nl

prefLabel

Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.

@en

Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.

@nl

P1433

Q42693496-5D2A04DE-51A9-4038-8CD6-480A03FD96F7

P1476

Q42693496-33237B3B-FDD1-4AC8-88DD-D6B4AE764DB2

P2093

Q42693496-385A912D-499C-4172-979C-383D0FBC4E6B

Q42693496-E703420F-16DC-407D-8B5F-20CC787EEB36

Q42693496-F241933C-390E-402B-8836-C22F5721E482

P2860

Q42693496-01ED4039-E13B-4746-BEFD-2F9DFDD8DEF2

Q42693496-09D59CA1-32C7-421D-9DC1-B2F82DECDDCE

Q42693496-125C3195-0B23-44EB-95CF-27D65961DDBB

Q42693496-12FF02C3-C54E-4BC5-9EFE-140E442AEE39

Q42693496-16EFBDB9-68AD-4866-82E3-76CFFEEF6162

Q42693496-21E4820A-E859-4575-8502-4E45FB6E0AC5

Q42693496-21F548B1-042D-4AA9-9D7A-D94463DF058F

Q42693496-23D541FF-D0C1-4BB7-8DBB-3D31C0A7304B

Q42693496-274D24A0-7CAB-421F-9B15-85BFA9B58178

Q42693496-2A3DE9BC-37AD-4740-9134-A6A0885F9168

P304

Q42693496-B9296DA2-7F48-4ABD-9097-F99F0D129AE0

P31

Q42693496-6073755A-2E45-47B5-93DC-2C82C9078CF4

P356

Q42693496-77E5BD43-E084-48FF-BE8F-51CE9DBFE439

P433

Q42693496-1F61705C-EB61-45F3-89B6-A5466F95A535

P478

Q42693496-AF6A6252-468F-42FF-94EF-E3FC603D4C16

P50

Q42693496-9F822CE4-32DD-4006-87E5-4835E2DB2FDB

Q42693496-B25F4B88-2ECC-4C63-9205-9641AEED1360

Q42693496-EDC9FB03-0183-4670-AC74-4D5C126B60F2

Q42693496-FDAB91FB-E103-4203-B2E2-42AF2C132102

P577

Q42693496-47F0B2DF-D5EA-4D4E-AAE7-2BB1FED35989

P5875

Q42693496-8A2DBFF5-14C0-43B0-ACDB-DFB8BC88E88E

P698

Q42693496-8D4E45DE-33BC-4448-B6F9-FAC9857D3821

P921

Q42693496-2B60FB46-CCAB-44E2-B864-0A76C0250FD3

P356

P1433

Human Molecular Genetics

P1476

Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.

@en

P2093

Mai Yamakawa

http://www.w3.org/2001/XMLSchema#string

Mariko Noda

http://www.w3.org/2001/XMLSchema#string

Takao Honda

http://www.w3.org/2001/XMLSchema#string

P2860

RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention

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

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

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

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

Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS

A simple method for displaying the hydropathic character of a protein

Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

Brn-1 and Brn-2 share crucial roles in the production and positioning of mouse neocortical neurons

P304

1630-1645

http://www.w3.org/2001/XMLSchema#string

P31

scholarly article

P356

10.1093/HMG/DDU576

http://www.w3.org/2001/XMLSchema#string

P433

6

http://www.w3.org/2001/XMLSchema#string

P478

24

http://www.w3.org/2001/XMLSchema#string

P50

Norihiro Suzuki

Kazunori Nakajima

Ken-ichiro Kubo

P577

2014-11-14T00:00:00Z

http://www.w3.org/2001/XMLSchema#dateTime

P5875

268336076

http://www.w3.org/2001/XMLSchema#string

P698

25398948

http://www.w3.org/2001/XMLSchema#string

P921

amyotrophic lateral sclerosis