Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7
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Ataxin-2 and its Drosophila homolog, ATX2, physically assemble with polyribosomesAtaxin-7 associates with microtubules and stabilizes the cytoskeletal network.Comparison of an expanded ataxia interactome with patient medical records reveals a relationship between macular degeneration and ataxiaBEST1 expression in the retinal pigment epithelium is modulated by OTX family membersZinc-finger domains of the transcriptional repressor KLF15 bind multiple sites in rhodopsin and IRBP promoters including the CRS-1 and G-rich repressor elementsA Drosophila homolog of the polyglutamine disease gene SCA2 is a dosage-sensitive regulator of actin filament formationStimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteinsGlutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction.Mouse models of polyglutamine diseases: review and data table. Part IThe promise and perils of HDAC inhibitors in neurodegenerationIntravitreal administration of HA-1077, a ROCK inhibitor, improves retinal function in a mouse model of huntington diseaseThe photoreceptor-specific nuclear receptor Nr2e3 interacts with Crx and exerts opposing effects on the transcription of rod versus cone genesHistone acetylation, acetyltransferases, and ataxia--alteration of histone acetylation and chromatin dynamics is implicated in the pathogenesis of polyglutamine-expansion disordersCTCF regulates ataxin-7 expression through promotion of a convergently transcribed, antisense noncoding RNA.PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function.Barrier to autointegration factor interacts with the cone-rod homeobox and represses its transactivation function.Kruppel-like factor 15, a zinc-finger transcriptional regulator, represses the rhodopsin and interphotoreceptor retinoid-binding protein promotersGenomic divergence between the migratory and stationary ecotypes of Atlantic cod.Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegenerationDesign of RNAi hairpins for mutation-specific silencing of ataxin-7 and correction of a SCA7 phenotype.Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration.Temporal retinal nerve fiber loss in patients with spinocerebellar ataxia type 1Transcription coactivators p300 and CBP are necessary for photoreceptor-specific chromatin organization and gene expression.Requirement for zebrafish ataxin-7 in differentiation of photoreceptors and cerebellar neurons.The SAGA histone deubiquitinase module controls yeast replicative lifespan via Sir2 interaction.Mouse retinal development: a dark horse model for systems biology researchElectroporation and RNA interference in the rodent retina in vivo and in vitro.RNA interference-based therapy for spinocerebellar ataxia type 7 retinal degeneration.Spinocerebellar ataxia type 7 associated with pigmentary retinal dystrophy.Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7.Preventing polyglutamine-induced activation of c-Jun delays neuronal dysfunction in a mouse model of SCA7 retinopathy.Pathways to motor incoordination: the inherited ataxias.A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseasesCrx activates opsin transcription by recruiting HAT-containing co-activators and promoting histone acetylation.PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically.Transcriptional signatures in Huntington's disease.Epigenetics in nucleotide repeat expansion disorders.Regulation of photoreceptor gene expression by Crx-associated transcription factor networkLentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins
P2860
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P2860
Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7
description
2001 nî lūn-bûn
@nan
2001 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年論文
@yue
2001年論文
@zh-hant
2001年論文
@zh-hk
2001年論文
@zh-mo
2001年論文
@zh-tw
2001年论文
@wuu
name
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@ast
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@en
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@nl
type
label
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@ast
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@en
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@nl
prefLabel
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@ast
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@en
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@nl
P2093
P3181
P1433
P1476
Polyglutamine-expanded ataxin- ...... rophy in a mouse model of SCA7
@en
P2093
A R La Spada
B L Sopher
D E Possin
J B Hurley
K L Koszdin
L J Ptácek
P304
P3181
P356
10.1016/S0896-6273(01)00422-6
P407
P577
2001-09-27T00:00:00Z