A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats.
about
Mouse models of polyglutamine diseases: review and data table. Part ISilencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic miceTherapeutic prospects for spinocerebellar ataxia type 2 and 3.RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph diseaseComputational analysis of calcium signaling and membrane electrophysiology in cerebellar Purkinje neurons associated with ataxia.Toward understanding Machado-Joseph diseaseAtaxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 modelsMachado-Joseph disease/spinocerebellar ataxia type 3.Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias.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.Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias.Mouse models of spinocerebellar ataxia type 3 (Machado-Joseph disease).Unravelling Endogenous MicroRNA System Dysfunction as a New Pathophysiological Mechanism in Machado-Joseph Disease.Mouse ataxin-3 functional knock-out model.Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).Overexpression of mutant ataxin-3 in mouse cerebellum induces ataxia and cerebellar neuropathology.Interaction between the AAA+ ATPase p97 and its cofactor ataxin3 in health and disease: Nucleotide-induced conformational changes regulate cofactor binding.Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3.
P2860
Q26853025-E541AD04-B28D-4612-BDB9-540600F47C87Q27324749-6EA866F9-BCA8-4293-9CBD-01D9AA4FD859Q30410969-6B3B3BBF-112D-466F-B8AA-27C41B7C60F6Q34075342-7253A03B-C28F-426A-840B-795221444D58Q34306449-7A5471E9-0E2C-42D4-AB17-63B035529242Q35837355-9648B000-F082-411A-8564-7FE77DD3BE24Q36583286-1EB6C79B-1A7A-435C-9E63-E6C08A7973F1Q36599588-53821242-5717-40B6-96A3-1B41AD342518Q37619548-A51D736E-1965-4CBA-9EE3-092A73A26B66Q37717405-E7FC1C47-B2D8-4E63-8816-3CF6A48F79F6Q37723704-9DBA89F7-61C5-45A2-B90D-5F0DC42C5A38Q37910368-732E9DC0-C63D-4168-A4BF-87D6D339E995Q37997384-673859C6-5FF1-4007-9963-EBA218ACBBB2Q38714908-9682FFCD-1C98-4DAF-B6A5-3E97F95FECBCQ39644424-C4509D41-86F1-4322-A0E8-0587AAFD4B53Q45310677-972AAAEC-493B-44CE-A84B-D658DE198431Q45349098-3C2635FA-D15A-48D6-B3F1-D1157D1DEFDEQ47758356-767DAFE8-B8BC-4735-B9A5-332BAFADE4EFQ51767599-6B66F6A6-C606-431D-86BF-51FF0BC72E09
P2860
A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats.
description
2009 nî lūn-bûn
@nan
2009年の論文
@ja
2009年学术文章
@wuu
2009年学术文章
@zh
2009年学术文章
@zh-cn
2009年学术文章
@zh-hans
2009年学术文章
@zh-my
2009年学术文章
@zh-sg
2009年學術文章
@yue
2009年學術文章
@zh-hant
name
A transgenic mouse model of sp ...... ic instability of CAG repeats.
@en
A transgenic mouse model of sp ...... ic instability of CAG repeats.
@nl
type
label
A transgenic mouse model of sp ...... ic instability of CAG repeats.
@en
A transgenic mouse model of sp ...... ic instability of CAG repeats.
@nl
prefLabel
A transgenic mouse model of sp ...... ic instability of CAG repeats.
@en
A transgenic mouse model of sp ...... ic instability of CAG repeats.
@nl
P2093
P50
P1476
A transgenic mouse model of sp ...... ic instability of CAG repeats.
@en
P2093
Franco Laccone
Hartwig Wolburg
Ina Schmitt
Samy Unser
Thorsten Schmidt
Ulrike Schumann
Ute Grasshoff
P304
P356
10.1016/J.NBD.2009.08.002
P577
2009-08-20T00:00:00Z