OMIA:000485-9544 : Huntington disease in Macaca mulatta (Rhesus monkey)

In other species: pig , sheep

Categories: Nervous system phene

Possibly relevant human trait(s) and/or gene(s)s (MIM numbers): 143100 (trait) , 613004 (gene)

Links to MONDO diseases:

Mendelian trait/disorder: yes

Considered a defect: yes

Key variant known: no

Species-specific description: Yang et al. (2008) developed "a transgenic model of HD in a rhesus macaque that expresses polyglutamine-expanded HTT". Resultant animals are genetically-modified organisms (GMO). Khampang et al. (2021) "report a novel stem cell model that replicates genome instability and CAG repeat expansion in in vitro derived HD monkey spermatogenic cells."

Genetic engineering: Yes - variants have been created artificially, e.g. by genetic engineering or gene editing
Have human generated variants been created, e.g. through genetic engineering and gene editing

Associated gene:

Symbol Description Species Chr Location OMIA gene details page Other Links
HTT huntingtin Macaca mulatta 5 NC_041758.1 (3190699..3356988) HTT Homologene, Ensembl , NCBI gene

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2023). OMIA:000485-9544: Online Mendelian Inheritance in Animals (OMIA) [dataset].


Note: the references are listed in reverse chronological order (from the most recent year to the earliest year), and alphabetically by first author within a year.

2023 Upadhayay, S., Jamwal, S., Kumar, P. :
Animal models of Huntington's disease and their applicability to novel drug discovery and development. Expert Opin Drug Discov :1-12, 2023. Pubmed reference: 37042034. DOI: 10.1080/17460441.2023.2201493.
Weiss, A.R., Liguore, W.A., Brandon, K., Wang, X., Liu, Z., Kroenke, C.D., McBride, J.L. :
Alterations of fractional anisotropy throughout cortico-basal ganglia gray matter in a macaque model of Huntington's Disease. Curr Res Neurobiol 4:100090, 2023. Pubmed reference: 37397804. DOI: 10.1016/j.crneur.2023.100090.
2021 Khampang, S., Parnpai, R., Mahikul, W., Easley, C.A., Cho, I.K., Chan, A.W.S. :
CAG repeat instability in embryonic stem cells and derivative spermatogenic cells of transgenic Huntington's disease monkey. J Assist Reprod Genet 38:1215-1229, 2021. Pubmed reference: 33611676. DOI: 10.1007/s10815-021-02106-3.
Yang, W., Chen, X., Li, S., Li, X.J. :
Genetically modified large animal models for investigating neurodegenerative diseases. Cell Biosci 11:218, 2021. Pubmed reference: 34933675. DOI: 10.1186/s13578-021-00729-8.
2020 Howland, D., Ellederova, Z., Aronin, N., Fernau, D., Gallagher, J., Taylor, A., Hennebold, J., Weiss, A.R., Gray-Edwards, H., McBride, J. :
Large animal models of Huntington's disease: What we have learned and where we need to go next. J Huntingtons Dis 9:201-216, 2020. Pubmed reference: 32925082. DOI: 10.3233/JHD-200425.
2019 Cho, I.K., Yang, B., Forest, C., Qian, L., Chan, A.W.S. :
Amelioration of Huntington's disease phenotype in astrocytes derived from iPSC-derived neural progenitor cells of Huntington's disease monkeys. PLoS One 14:e0214156, 2019. Pubmed reference: 30897183. DOI: 10.1371/journal.pone.0214156.
Clever, F., Cho, I.K., Yang, J., Chan, A. :
Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington's Disease Monkeys. J Huntingtons Dis , 2019. Pubmed reference: 31561381. DOI: 10.3233/JHD-190359.
2018 Snyder, B.R., Chan, A.W.S. :
Progress in developing transgenic monkey model for Huntington's disease. J Neural Transm (Vienna) 125:401-417, 2018. Pubmed reference: 29127484. DOI: 10.1007/s00702-017-1803-y.
2017 Meng, Y., Jiang, J., Bachevalier, J., Zhang, X., Chan, A.W. :
Developmental Whole Brain White Matter Alterations in Transgenic Huntington's Disease Monkey. Sci Rep 7:379, 2017. Pubmed reference: 28336929. DOI: 10.1038/s41598-017-00381-8.
2015 Chang, R., Liu, X., Li, S., Li, X.J. :
Transgenic animal models for study of the pathogenesis of Huntington's disease and therapy. Drug Des Devel Ther 9:2179-88, 2015. Pubmed reference: 25931812. DOI: 10.2147/DDDT.S58470.
Li, X.J., Li, S. :
Large Animal Models of Huntington's Disease. Curr Top Behav Neurosci 22:149-60, 2015. Pubmed reference: 24048953. DOI: 10.1007/7854_2013_246.
2014 Chan, A.W., Xu, Y., Jiang, J., Rahim, T., Zhao, D., Kocerha, J., Chi, T., Moran, S., Engelhardt, H., Larkin, K., Neumann, A., Cheng, H., Li, C., Nelson, K., Banta, H., Zola, S.M., Villinger, F., Yang, J., Testa, C.M., Mao, H., Zhang, X., Bachevalier, J. :
A two years longitudinal study of a transgenic Huntington disease monkey. BMC Neurosci 15:36, 2014. Pubmed reference: 24581271. DOI: 10.1186/1471-2202-15-36.
2013 Laowtammathron, C., Chan, A.W. :
Pluripotent hybrid stem cells from transgenic Huntington's disease monkey. Methods Mol Biol 1010:61-77, 2013. Pubmed reference: 23754219. DOI: 10.1007/978-1-62703-411-1_5.
2008 Yang, S.H., Cheng, P.H., Banta, H., Piotrowska-Nitsche, K., Yang, J.J., Cheng, E.C., Snyder, B., Larkin, K., Liu, J., Orkin, J., Fang, Z.H., Smith, Y., Bachevalier, J., Zola, S.M., Li, S.H., Li, X.J., Chan, A.W. :
Towards a transgenic model of Huntington's disease in a non-human primate. Nature 453:921-4, 2008. Pubmed reference: 18488016. DOI: 10.1038/nature06975.
1997 Emerich, D.F., Winn, S.R., Hantraye, P.M., Peschanski, M., Chen, E.Y., Chu, Y., McDermott, P., Baetge, E.E., Kordower, J.H. :
Protective effect of encapsulated cells producing neurotrophic factor CNTF in a monkey model of Huntington's disease. Nature 386:395-9, 1997. Pubmed reference: 9121555. DOI: 10.1038/386395a0.

Edit History

  • Created by Frank Nicholas on 30 Sep 2019
  • Changed by Frank Nicholas on 30 Sep 2019
  • Changed by Imke Tammen2 on 08 Apr 2021
  • Changed by Imke Tammen2 on 09 Jan 2022
  • Changed by Imke Tammen2 on 10 Dec 2023