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在“Elsevier”中，命中：25条，耗时：0.0459807 秒

1.DRPLA transgenic mouse substrains carrying single copy of full-length mutant human DRPLA gene with variable sizes of expanded CAG repeats exhibit CAG repeat length- and age-dependent changes in behavioral abnormalities and gene expression profiles

作者：Kazushi Suzuki^a ; ¹ ; Jiayi Zhou^a ; ¹ ; ² ; Toshiya Sato^b ; Keizo Takao^e ; ^f ; ^g ; Tsuyoshi Miyagawa^e ; ^f ; ^g ; Mutsuo Oyake^c ; ³ ; Mitunori Yamada^d ; Hitoshi Takahashi^d ; Yuji Takahashi^a ; Jun Goto^a ; Shoji Tsuji^a ; ^{tsuji@m.u-tokyo.ac.jp}

关键词：ANOVA ; analysis of variance ; CDK ; cyclin-dependent kinase ; CREB ; cAMP response element-binding protein ; DRPLA ; dentatorubral-pallidoluysian atrophy ; GO ; gene ontology ; KO ; knock-out ; MAPK ; mitogen-activated protein kinase ; MJD ; Machado&ndash ; Joseph diseas

刊名：Neurobiology of Disease

出版年：2012

2.Amelioration of disabling myoclonus in a case of DRPLA by levetiracetam

作者：Katsuhiro Kobayashi^a ; ^{k_koba@md.okayama-u.ac.jp} ; Akihito Takeuchi^a ; Makio Oka^a ; Mari Akiyama^a ; ^b ; Yoko Ohtsuka^a

关键词：Dentatorubral-pallidoluysian atrophy ; Myoclonus ; Negative myoclonus ; Levetiracetam ; Zonisamide

刊名：Brain and Development

出版年：2012

3.Autophagy and polyglutamine diseases

作者：Maria Jimenez-Sanchez¹ ; Frances Thomson¹ ; Eszter Zavodszky¹ ; David C. Rubinsztein ; ^{dcr1000@hermes.cam.ac.uk}

关键词：HD ; Huntington's disease ; SCA ; spinocerebellar ataxia ; DRPLA ; Denatorubral-pallidoluysian atrophy ; SBMA ; spinal and bulbar muscular atropy ; Htt ; Huntingtin ; UPS ; ubiquitin&ndash ; proteasome system ; HDL-2 ; Huntington's disease-like 2 ; IBs ; inclusion bodies

刊名：Progress in Neurobiology

出版年：2012

4.Toxic effects of expanded ataxin-1 involve mechanical instability of the nuclear membrane

作者：Lisa Mapelli^a ; ¹ ; ² ; Claudio Canale^b ; ¹ ; Daniela Pesci^c ; ¹ ; Stefania Averaimo^a ; Fabiana Guizzardi^a ; ³ ; Valentina Fortunati^c ; Laura Falasca^d ; Mauro Piacentini^c ; ^d ; Alessandra Gliozzi^e ; ^f ; Annalisa Relini^e ; ^f ; ⁴ ; Michele Mazzanti^a ; ⁴ ; Carla Jodice^c ; ^f ; ⁴ ; ^{carla.jodice@uniroma2.it}

关键词：SCA1 ; spinocerebellar ataxia type 1 ; ATXN1 ; ataxin 1 ; polyglutamine ; polyQ ; SBMA ; spinal bulbar muscular atrophy ; HD ; Huntington disease ; DRPLA ; dentatorubropallidoluysian atrophy ; NLS ; nuclear localization signal ; RBM17 ; RNA-binding motif protein 17 ; AFM

刊名：Biochimica et Biophysica Acta (BBA)/Molecular Basis of Disease

出版年：2012

5.Analysis of spinocerebellar ataxias due to expanded triplet repeats in Greek patients with cerebellar ataxia

作者：Georgios Koutsis^a ; ^c ; ^{gkoutsi2@otenet.gr} ; Sally Pemble^b ; Mary G. Sweeney^b ; Reema Paudel^a ; Nicholas W. Wood^a ; Marios Panas^c ; Athina Kladi^c ; Henry Houlden^a

关键词：Spinocerebellar ataxia ; SCA ; Autosomal dominant cerebellar ataxia ; Expanded triplet repeat disorders ; SCA1 ; SCA2 ; SCA7

刊名：Journal of the Neurological Sciences

出版年：2012

6.Differences in evoked potential characteristics between DRPLA patients and patients with progressive myoclonic epilepsy: preliminary findings indicating usefulness for differential diagnosis

作者：Kasai ; Kiyoto ; Onuma ; Teiichi ; Kato ; Masaaki ; Kato ; Takeji ; Takeya ; Jun ; Sekimoto ; Masanori ; et. al.

关键词：Dentatorubral-pallidoluysian atrophy (DRPLA) ; Evoked potentials ; Somatosensory evoked potentials (SEPs) ; Brainstem auditory evoked responses (BAERs) ; Progressive myoclonic epilepsy ; Unverricht-Lundborg disease

刊名：Epilepsy Research

出版年：1999

7.Neuropathological analysis of the brainstem and cerebral cortex lesions on epileptogenesis in hereditary dentatorubral-pallidoluysian atrophy

作者：Masaharu Hayashi ; Satoko Kumada ; Kei Shioda ; Ryo Fukatsu

关键词：Dentatorubral-pallidoluysian atrophy ; Immunohistochemistry ; Cerebral cortex ; Brainstem ; Calcium-binding protein

刊名：Brain and Development

出版年：2007

8.Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases

作者：Jiou Wang ; Elizabeth Martin ; Victoria Gonzales ; David R. Borchelt ; Michael K. Lee

关键词：Neurodegeneration ; Aging ; Hsp25 ;

//www.sciencedirect.com/scidirimg/entities/204e.gif" alt="greek small letter alpha" title="greek small letter alpha" border="0">B-crystallin

刊名：Neurobiology of Aging

出版年：2008

9.Proton magnetic resonance spectroscopy on childhood-onset dentatorubral-pallidoluysian atrophy (DRPLA)

作者：Miyazaki ; Masahito ; Hashimoto ; Toshiaki ; Yoneda ; Yoshihiro ; Tayama ; Masanobu ; Harada ; Masafumi ; et. al.

关键词：¹H-MRS ; Progressive myoclonus epilepsy ; DRPLA ; Childhood onset ; NAA/Cr

刊名：Brain and Development

出版年：1996

10.Common mitochondrial DNA and POLG1 mutations are rare in the Chinese patients with adult-onset ataxia on Taiwan

作者：Yi-Chung Lee ; Yi-Chun Lu ; Ming-Hon Chang ; Bing-Wen Soong

关键词：SCA ; Ataxia ; POLG1 ; POLG ; Mitochondria ; MIRAS

刊名：Journal of the Neurological Sciences

出版年：2007

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