Literature DB >> 29856016

Mouse Models of Huntington's Disease.

Pamela P Farshim1, Gillian P Bates2.   

Abstract

The identification of the mutation causing Huntington's disease (HD) has led to the generation of a large number of mouse models. These models are used to further enhance our understanding of the mechanisms underlying the disease, as well as investigating and identifying therapeutic targets for this disorder. Here we review the transgenic, knock-in mice commonly used to model HD, as well those that have been generated to study specific disease mechanisms. We then provide a brief overview of the importance of standardizing the use of HD mice and describe brief protocols used for genotyping the mouse models used within the Bates Laboratory.

Entities:  

Keywords:  CAG repeat; Huntingtin; Huntington’s disease; Inbred strain; Mouse models; N-terminal fragment; Polyglutamine; Transgenic; knock-in

Mesh:

Substances:

Year:  2018        PMID: 29856016     DOI: 10.1007/978-1-4939-7825-0_6

Source DB:  PubMed          Journal:  Methods Mol Biol        ISSN: 1064-3745


  22 in total

Review 1.  Spermatozoan Metabolism as a Non-Traditional Model for the Study of Huntington's Disease.

Authors:  Meghan Lawlor; Michal Zigo; Karl Kerns; In Ki Cho; Charles A Easley Iv; Peter Sutovsky
Journal:  Int J Mol Sci       Date:  2022-06-28       Impact factor: 6.208

2.  Uninterrupted CAG repeat drives striatum-selective transcriptionopathy and nuclear pathogenesis in human Huntingtin BAC mice.

Authors:  Xiaofeng Gu; Jeffrey Richman; Peter Langfelder; Nan Wang; Shasha Zhang; Monica Bañez-Coronel; Huei-Bin Wang; Lucia Yang; Lalini Ramanathan; Linna Deng; Chang Sin Park; Christopher R Choi; Jeffrey P Cantle; Fuying Gao; Michelle Gray; Giovanni Coppola; Gillian P Bates; Laura P W Ranum; Steve Horvath; Christopher S Colwell; X William Yang
Journal:  Neuron       Date:  2022-02-02       Impact factor: 18.688

3.  Truncation of mutant huntingtin in knock-in mice demonstrates exon1 huntingtin is a key pathogenic form.

Authors:  Huiming Yang; Su Yang; Liang Jing; Luoxiu Huang; Luxiao Chen; Xianxian Zhao; Weili Yang; Yongcheng Pan; Peng Yin; Zhaohui S Qin; Beisha Tang; Shihua Li; Xiao-Jiang Li
Journal:  Nat Commun       Date:  2020-05-22       Impact factor: 14.919

4.  Genetic deletion of S6k1 does not rescue the phenotypic deficits observed in the R6/2 mouse model of Huntington's disease.

Authors:  Elaine E Irvine; Loukia Katsouri; Florian Plattner; Hind Al-Qassab; Rand Al-Nackkash; Gillian P Bates; Dominic J Withers
Journal:  Sci Rep       Date:  2019-11-06       Impact factor: 4.379

5.  Genetic Screen in Adult Drosophila Reveals That dCBP Depletion in Glial Cells Mitigates Huntington Disease Pathology through a Foxo-Dependent Pathway.

Authors:  Elodie Martin; Raheleh Heidari; Véronique Monnier; Hervé Tricoire
Journal:  Int J Mol Sci       Date:  2021-04-09       Impact factor: 5.923

6.  Targeted Genetic Reduction of Mutant Huntingtin Lessens Cardiac Pathology in the BACHD Mouse Model of Huntington's Disease.

Authors:  Saemi Park; Shu Hon Christopher Luk; Raj S Bains; Daniel S Whittaker; Emily Chiem; Maria C Jordan; Kenneth P Roos; Cristina A Ghiani; Christopher S Colwell
Journal:  Front Cardiovasc Med       Date:  2021-12-24

7.  In Vivo Expression of Reprogramming Factor OCT4 Ameliorates Myelination Deficits and Induces Striatal Neuroprotection in Huntington's Disease.

Authors:  Ji-Hea Yu; Bae-Geun Nam; Min-Gi Kim; Soonil Pyo; Jung-Hwa Seo; Sung-Rae Cho
Journal:  Genes (Basel)       Date:  2021-05-10       Impact factor: 4.096

8.  Systematic metabolic analysis of potential target, therapeutic drug, diagnostic method and animal model applicability in three neurodegenerative diseases.

Authors:  Wen-Xing Li; Gong-Hua Li; Xin Tong; Peng-Peng Yang; Jing-Fei Huang; Lin Xu; Shao-Xing Dai
Journal:  Aging (Albany NY)       Date:  2020-05-27       Impact factor: 5.682

9.  Abnormal Brain Development in Huntington' Disease Is Recapitulated in the zQ175 Knock-In Mouse Model.

Authors:  Chuangchuang Zhang; Qian Wu; Hongshuai Liu; Liam Cheng; Zhipeng Hou; Susumu Mori; Jun Hua; Christopher A Ross; Jiangyang Zhang; Peggy C Nopoulos; Wenzhen Duan
Journal:  Cereb Cortex Commun       Date:  2020-08-05

Review 10.  Large Animal Models of Huntington's Disease: What We Have Learned and Where We Need to Go Next.

Authors:  David Howland; Zdenka Ellederova; Neil Aronin; Deborah Fernau; Jill Gallagher; Amanda Taylor; Jon Hennebold; Alison R Weiss; Heather Gray-Edwards; Jodi McBride
Journal:  J Huntingtons Dis       Date:  2020
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