about
Neurodegenerative diseases in a dish: the promise of iPSC technology in disease modeling and therapeutic discoveryPatterns of motor signs in spinocerebellar ataxia type 3 at the start of follow-up in a reference unit.Deubiquitinases (DUBs) and DUB inhibitors: a patent reviewStructure prediction of polyglutamine disease proteins: comparison of methods.Trinucleotide repeats: a structural perspectiveEvaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse ModelsAllele-selective inhibition of mutant atrophin-1 expression by duplex and single-stranded RNAs.An optimal ubiquitin-proteasome pathway in the nervous system: the role of deubiquitinating enzymes.Using membrane-targeted green fluorescent protein to monitor neurotoxic protein-dependent degeneration of Drosophila eyesUbiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23Characterization of the conformational fluctuations in the Josephin domain of ataxin-3.Inactivation of PNKP by mutant ATXN3 triggers apoptosis by activating the DNA damage-response pathway in SCA3Two novel SNPs in ATXN3 3' UTR may decrease age at onset of SCA3/MJD in Chinese patients.RNA-binding protein misregulation in microsatellite expansion disordersEpidemiological, clinical, and molecular characterization of Cuban families with spinocerebellar ataxia type 3/Machado-Joseph disease.Alleviating neurodegeneration in Drosophila models of PolyQ diseases.Human pluripotent stem cells: applications and challenges in neurological diseases.The Machado-Joseph Disease Deubiquitinase Ataxin-3 Regulates the Stability and Apoptotic Function of p53The deubiquitinase ataxin-3 requires Rad23 and DnaJ-1 for its neuroprotective role in Drosophila melanogaster.The 2.2-Angstrom resolution crystal structure of the carboxy-terminal region of ataxin-3.JosD1, a membrane-targeted deubiquitinating enzyme, is activated by ubiquitination and regulates membrane dynamics, cell motility, and endocytosisFar-infrared radiation protects viability in a cell model of Spinocerebellar Ataxia by preventing polyQ protein accumulation and improving mitochondrial functionRNA duplexes with abasic substitutions are potent and allele-selective inhibitors of huntingtin and ataxin-3 expression.Toward RNAi therapy for the polyglutamine disease Machado-Joseph diseaseSilencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic micess-siRNAs allele selectively inhibit ataxin-3 expression: multiple mechanisms for an alternative gene silencing strategy.Ubiquitination regulates the neuroprotective function of the deubiquitinase ataxin-3 in vivo.Compound heterozygous intermediate MJD alleles cause cerebellar ataxia with sensory neuropathy.CAG repeat length does not associate with the rate of cerebellar degeneration in spinocerebellar ataxia type 3.Allele-selective inhibition of expression of huntingtin and ataxin-3 by RNA duplexes containing unlocked nucleic acid substitutions.Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias.The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease.Genetic counseling and presymptomatic testing programs for Machado-Joseph Disease: lessons from Brazil and Portugal.Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease.Transgenic Monkey Model of the Polyglutamine Diseases Recapitulating Progressive Neurological Symptoms.Disease modeling and drug screening for neurological diseases using human induced pluripotent stem cells.Deubiquitylating enzymes and their emerging role in plant biology.Peripheral neuropathy and parkinsonism: a large clinical and pathogenic spectrum.DUBs, the regulation of cell identity and disease.A randomized, phase 2 clinical trial of lithium carbonate in Machado-Joseph disease.
P2860
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P2860
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
Toward understanding Machado-Joseph disease
@ast
Toward understanding Machado-Joseph disease
@en
type
label
Toward understanding Machado-Joseph disease
@ast
Toward understanding Machado-Joseph disease
@en
prefLabel
Toward understanding Machado-Joseph disease
@ast
Toward understanding Machado-Joseph disease
@en
P2860
P1476
Toward understanding Machado-Joseph disease
@en
P2093
Henry L Paulson
Maria do Carmo Costa
P2860
P304
P356
10.1016/J.PNEUROBIO.2011.11.006
P577
2011-11-23T00:00:00Z