Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Article
  • Published:

Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues

Nature Genetics volume 5, pages 259–265 (1993)Cite this article

Abstract

We have used RNA in situ hybridization to study the regional expression of the Huntington's disease gene (HD) and its rat homologue in brain and selected nonneural tissues. The HD transcript was expressed throughout the brain in both rat and human, especially in the neurons of the dentate gyrus and pyramidal neurons of the hippocampal formation, cerebellar granule cell layer, cerebellar Purkinje cells and pontine nuclei. Other brain areas expressed lower levels of the HD transcript without pronounced regional differences. Neuronal expression predominated over glial expression in all regions. HD mRNA was also expressed in colon, liver, pancreas and testes. The regional specificity of neuropathology in HD, which is most prominent in the basal ganglia, thus cannot be accounted for by the pattern of expression of HD.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Similar content being viewed by others

References

  1. Folstein, S. In Huntington's Disease: A Disorder of Families (Johns Hopkins, Baltimore, 1989).

    Google Scholar 

  2. Quarrel, O. & Harper, P.S. In Huntington's Disease (ed. Harper, P.S.) 37–80 (W.B. Saunders, Philadelphia, 1991).

    Google Scholar 

  3. Morris, M. In Huntington's Disease (ed. Harper, P.S.) 81–126 (W.B. Saunders, Philadelphia, 1991).

    Google Scholar 

  4. Quarrel, O. In Huntington's Disease (ed. Harper, P.S.) 141–178 (W.B. Saunders, Philadelphia, 1991).

    Google Scholar 

  5. Beal, M.F. Does impairment of energy metabolism result in excitoxic neuronal death in neurodegenerative illnesses?. Ann. Neurol. 31, 119–130 (1992).

    Article  CAS  Google Scholar 

  6. Huntington's Disease Collaborative Research Group. A novel gene contianing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72, 971–983 (1993).

    Article  Google Scholar 

  7. DiFiglia, M. Excitotoxic injury of the neostriatum: a model for Huntington's disease. Trends Neurosci. 13, 286–289 (1990).

    Article  CAS  Google Scholar 

  8. Wexler, N.S. et al. Homozygotes for Huntington's disease. Nature 326, 194–197 (1987).

    Article  CAS  Google Scholar 

  9. Myers, R.H. et al. Homozygote for Huntington Disease. Am. J. hum. Genet. 45, 615–618 (1989).

    CAS  PubMed  PubMed Central  Google Scholar 

  10. Sabourin, L.A. et al. Effect of the myotonic dystrophy (DM) mutation on mRNA levels of the DM gene. Nature Genet. 4, 233–238 (1993).

    Article  Google Scholar 

  11. Fu, Y.H. et al. Decreased expression of myotonin-protein kinase messenger RNA and protein in adult form of myotonic dystrophy. Science 260, 235–238 (1993).

    Article  CAS  Google Scholar 

  12. Rosen, D.R. et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362, 59–62 (1993).

    Article  CAS  Google Scholar 

  13. Ferrante, R.J. et al. Selective sparing of a class of striatal neurons in Huntington's disease. Science 230, 561–563 (1985).

    Article  CAS  Google Scholar 

  14. Dawbarn, D., DeQuidt, M.E. & Emson, P.C. Survival of basal ganglia neuropeptide Y-somatostatin neurons in Huntington's disease. Brain Res. 340, 251–260 (1985).

    Article  CAS  Google Scholar 

  15. Ferrante, R.J., Beal, M.F., Kowall, N.W., Richardson, E.P. & Martin, J.B. Sparing of acetylcholinesterase-containing striatal neurons in Huntington's disease. Brain Res. 411, 162–166 (1987).

    Article  CAS  Google Scholar 

  16. Hirsch, E.C., Graybiel, A.M., Hersh, L.B., Duyckaerts, C. & Agid, Y. Striosomes and extrastriosomal matrix contain different amounts of immunoreactive choline acetyttransferase in the human striatum. Neurosci. Lett. 96, 145–150 (1989).

    Article  CAS  Google Scholar 

  17. Herrington, K.M. & Kowall, N.W. Parvalbumin immunoreactive neurons resist degeneration in Huntington's disease striatum. J. Neruopath. Exp. Neurol. 50, 309 (1991).

    Google Scholar 

  18. Reiner, A. et al. Differential loss of striatal projection neurons in Huntington disease. Proc. natn. Acad. Sci. U.S.A. 85, 5733–5737 (1988).

    Article  CAS  Google Scholar 

  19. Albin, R.L. et al. Abnormalities of striatal projection neurons and N-methyl-D-aspartate receptors in presymptomatic Huntington's disease. New Engl. J. Med. 322, 1293–1298 (1990).

    Article  CAS  Google Scholar 

  20. Sambrook, J., Fritsch, E.F. & Maniatis, T. In Molecular Cloning: A Laboratory Manual (Cold Spring Harbor Laboratory Press, New York, 1989).

    Google Scholar 

  21. Devereux, J., Haeberli, P. & Smithies, O. A comprehensive set of sequence analysis programs for the VAX. Nucl. Acids Res. 12, 387–395 (1984).

    Article  CAS  Google Scholar 

  22. Strong, T.V., Boehm, K. & Collins, F.S. Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastro-intestinal tract by insitu hybridization. J. clin. Invest. (in the press).

  23. Paxinos, G. & Watson, C. In The Rat Brain in Stereotaxic Coordinates (Academic Press, Orlando, 1986).

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Molecular Medicine and Genetics, Department of Internal Medicine, 4570 MSRB II

    Theresa V. Strong, Danilo A. Tagle, Lawrence W. Elmer, Karina Boehm, Manju Swaroop & Francis S. Collins

  2. Department of Human Genetics, University of Michigan, Ann Arbor, Michigan, 48109, USA

    Theresa V. Strong, Danilo A. Tagle & Francis S. Collins

  3. Cell and Molecular Biology Program, University of Michigan, Ann Arbor, Michigan, 48109, USA

    John M. Valdes

  4. Department of Neurology, Neuroscience Laboratory Building, University of Michigan, 1103 E. Huron, Ann Arbor, Michigan, 48104, USA

    Lawrence W. Elmer, Kevin W. Kaatz & Roger L. Albin

  5. National Center for Human Genome Research, NIH, Bethesda, MD, 20892, USA

    Francis S. Collins

Authors
  1. Theresa V. Strong
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Danilo A. Tagle
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. John M. Valdes
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Lawrence W. Elmer
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Karina Boehm
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Manju Swaroop
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Kevin W. Kaatz
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Francis S. Collins
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Roger L. Albin
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Strong, T., Tagle, D., Valdes, J. et al. Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues. Nat Genet 5, 259–265 (1993). https://doi.org/10.1038/ng1193-259

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ng1193-259

This article is cited by

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing