相关参考文献
注意：仅列出部分参考文献，下载原文获取全部文献信息。Motor deficits associated with Huntington's disease occur in the absence of striatal degeneration in BACHD transgenic mice
Susanna Mantovani et al.
HUMAN MOLECULAR GENETICS (2016)
HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity
Virginia B. Mattis et al.
HUMAN MOLECULAR GENETICS (2015)
Altered excitatory and inhibitory inputs to striatal medium-sized spiny neurons and cortical pyramidal neurons in the Q175 mouse model of Huntington's disease
Tim Indersmitten et al.
JOURNAL OF NEUROPHYSIOLOGY (2015)
Aβ reduction in BACE1 heterozygous null 5XFAD mice is associated with transgenic APP level
Katherine R. Sadleir et al.
MOLECULAR NEURODEGENERATION (2015)
Therapeutic Advances in Huntington's Disease
Kathleen M. Shannon et al.
MOVEMENT DISORDERS (2015)
Progressive axonal transport and synaptic protein changes correlate with behavioral and neuropathological abnormalities in the heterozygous Q175 KI mouse model of Huntington's disease
Gaynor A. Smith et al.
HUMAN MOLECULAR GENETICS (2014)
Multisource Ascertainment of Huntington Disease in Canada: Prevalence and Population at Risk
Emily R. Fisher et al.
MOVEMENT DISORDERS (2014)
A fully humanized transgenic mouse model of Huntington disease
Amber L. Southwell et al.
HUMAN MOLECULAR GENETICS (2013)
Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records
Stephen J. W. Evans et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2013)
Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data
Sarah J. Tabrizi et al.
LANCET NEUROLOGY (2013)
Choosing an animal model for the study of Huntington's disease
Mahmoud A. Pouladi et al.
NATURE REVIEWS NEUROSCIENCE (2013)
A touch screen-automated cognitive test battery reveals impaired attention, memory abnormalities, and increased response inhibition in the TgCRND8 mouse model of Alzheimer's disease
Carola Romberg et al.
NEUROBIOLOGY OF AGING (2013)
Mismatch Repair Genes Mlh1 and Mlh3 Modify CAG Instability in Huntington's Disease Mice: Genome-Wide and Candidate Approaches
Ricardo Mouro Pinto et al.
PLOS GENETICS (2013)
Induced Pluripotent Stem Cells from Patients with Huntington's Disease Show CAG-Repeat-Expansion-Associated Phenotypes
CELL STEM CELL (2012)
Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice
Mahmoud A. Pouladi et al.
HUMAN MOLECULAR GENETICS (2012)
Comprehensive Behavioral and Molecular Characterization of a New Knock-In Mouse Model of Huntington's Disease: zQ175
Liliana B. Menalled et al.
PLOS ONE (2012)
Characterization of Neurophysiological and Behavioral Changes, MRI Brain Volumetry and 1H MRS in zQ175 Knock-In Mouse Model of Huntington's Disease
Taneli Heikkinen et al.
PLOS ONE (2012)
Huntington's disease: from molecular pathogenesis to clinical treatment
Christopher A. Ross et al.
LANCET NEUROLOGY (2011)
Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease
Jeffrey B. Carroll et al.
MOLECULAR NEURODEGENERATION (2011)
Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice
Rocky G. Gogliotti et al.
NEUROBIOLOGY OF DISEASE (2011)
Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease
Jeffrey B. Carroll et al.
NEUROBIOLOGY OF DISEASE (2011)
Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Chiara Zuccato et al.
PHYSIOLOGICAL REVIEWS (2010)
Huntington's disease
Marianne J. U. Novak et al.
BMJ-BRITISH MEDICAL JOURNAL (2010)
Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin
Mahmoud A. Pouladi et al.
BRAIN (2009)
Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease
Amber L. Southwell et al.
JOURNAL OF NEUROSCIENCE (2009)
Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin
Chuan-En Wang et al.
JOURNAL OF CELL BIOLOGY (2008)
Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice
Michelle Gray et al.
JOURNAL OF NEUROSCIENCE (2008)
Rodent genetic models of Huntington disease
Mary Y. Heng et al.
NEUROBIOLOGY OF DISEASE (2008)
Huntington disease models and human neuropathology: similarities and differences
Jean Paul G. Vonsattel
ACTA NEUROPATHOLOGICA (2008)
Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage
Alexandre Kuhn et al.
HUMAN MOLECULAR GENETICS (2007)
Generation of a germ cell-specific mouse transgenic Cre line, Vasa-Cre
Teresa Gallardo et al.
GENESIS (2007)
Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain
Jeremy M. Van Raamsdonk et al.
NEUROBIOLOGY OF DISEASE (2007)
The operant serial implicit learning task reveals early onset motor learning deficits in the Hdh(Q92) knock-in mouse model of Huntington's disease
Rebecca C. Trueman et al.
EUROPEAN JOURNAL OF NEUROSCIENCE (2007)
Testicular degeneration in Huntington disease
Jeremy M. Van Raamsdonk et al.
NEUROBIOLOGY OF DISEASE (2007)
Regional and cellular gene expression changes in human Huntington's disease brain
A Hodges et al.
HUMAN MOLECULAR GENETICS (2006)
Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models
RK Graham et al.
NEUROBIOLOGY OF DISEASE (2006)
Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease
E Papalexi et al.
EUROPEAN JOURNAL OF NEUROSCIENCE (2005)
Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease
JM Van Raamsdonk et al.
JOURNAL OF NEUROSCIENCE (2005)
Plasma testosterone in male patients with Huntington's disease: Relations to severity of illness and dementia
M Markianos et al.
ANNALS OF NEUROLOGY (2005)
GABA transporter deficiency causes tremor, ataxia, nervousness, and increased GABA-induced tonic conductance in cerebellum
CS Chiu et al.
JOURNAL OF NEUROSCIENCE (2005)
Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats
LB Menalled et al.
JOURNAL OF COMPARATIVE NEUROLOGY (2003)
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
EJ Slow et al.
HUMAN MOLECULAR GENETICS (2003)
Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course
F Squitieri et al.
BRAIN (2003)
The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin
W Auerbach et al.
HUMAN MOLECULAR GENETICS (2001)
Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo
BR Leavitt et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2001)
Neurological abnormalities in a knock-in mouse model of Huntington's disease
CH Lin et al.
HUMAN MOLECULAR GENETICS (2001)
Cre-mediated germline mosaicism: a method allowing rapid generation of several alleles of a target gene
Martin Holzenberger et al.
NUCLEIC ACIDS RESEARCH (2000)
分享论文
