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Analysis of gene expression related to adrenal physiological functions in Huntington’s disease mouse model Cover

Analysis of gene expression related to adrenal physiological functions in Huntington’s disease mouse model

Open Access
|Dec 2025

References

  1. Huntington G. On chorea. George Huntington, M.D. J Neuropsychiatry Clin Neurosci. 2003;15(1):109-12; DOI:10.1176/jnp.15.1.109.
  2. Dayalu P, Albin RL. Huntington disease: pathogenesis and treatment. Neurol Clin. 2015;33(1):101-14; DOI:10.1016/j.ncl.2014.09.003.
  3. Paulsen JS, Nehl C, Hoth KF, Kanz JE, Benjamin M, Conybeare R, McDo­well B, Turner B. Depression and stages of Huntington’s disease. J Neuropsychiatry Clin Neurosci. 2005;17(4):496-502; DOI:10.1176/jnp.17.4.496.
  4. Gusella JF, Wexler NS, Conneally PM, Naylor SL, Anderson MA, Tanzi RE, Watkins PC, Ottina K, Wallace MR, Sakaguchi AY, Young AB, Sho­ulson I, Bonilla E, Martin JB. A polymorphic DNA marker genetical­ly linked to Huntington’s disease. Nature. 1983;306(5940):234-8; DOI:10.1038/306234a0.
  5. MacDonald ME, Ambrose CM, Duyao MP, Myers RH, Lin C, Srinidhi L, Barnes G, Taylor SA, James M, Groot N, MacFarlane H, Jenkins B, An­derson MA, Wexler NS, Gusella JF, Bates GP, Baxendale S, Hummerich H, Kirby S, North M, Youngman S, Mott R, Zehetner G, Sedlacek Z, Poustka A, Frischauf A-M, Lehrach H, Buckler AJ, Church D, Doucette-Stamm L, O’Donovan MC, Riba-Ramirez L, Shah M, Stanton VP, Strobel SA, Draths KM, Wales JL, Dervan P, Housman DE, Altherr M, Shiang R, Thompson L, Fielder T, Wasmuth JJ, Tagle D, Valdes J, Elmer L, Allard M, Castilla L, Swaroop M, Blanchard K, Collins FS, Snell R, Holloway T, Gillespie K, Datson N, Shaw D, Harper P. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromo­somes. The Huntington’s Disease Collaborative Research Group. Cell. 1993;72(6):971-83; DOI:10.1016/0092-8674(93)90585-e.
  6. Sathasivam K, Hobbs C, Turmaine M, Mangiarini L, Mahal A, Bertaux F, Wanker EE, Doherty P, Davies SW, Bates GP. Formation of polygluta­mine inclusions in non-CNS tissue. Hum Mol Genet. 1999;8(5):813-22; DOI:10.1093/hmg/8.5.813.
  7. Martinez-Ramirez C, Baraibar AM, Nanclares C, Mendez-Lopez I, Go­mez A, Munoz MP, de Diego AMG, Gandia L, Casarejos MJ, Garcia AG. Altered excitability and exocytosis in chromaffin cells from the R6/1 mouse model of Huntington’s disease is linked to over-expression of mutated huntingtin. J Neurochem. 2018;147(4):454-76; DOI:10.1111/jnc.14585.
  8. Olechnowicz A, Blatkiewicz M, Jopek K, Isalan M, Mielcarek M, Rucinski M. Deregulated transcriptome as a platform for adrenal Huntington’s di­sease-related pathology. Int J Mol Sci. 2024;25(4):2176; DOI:10.3390/ijms25042176.
  9. Aziz NA, Pijl H, Frolich M, van der Graaf AW, Roelfsema F, Roos RA. In­creased hypothalamic-pituitary-adrenal axis activity in Huntington’s disease. J Clin Endocrinol Metab. 2009;94(4):1223-8; DOI:10.1210/jc.2008-2543.
  10. Saleh N, Moutereau S, Durr A, Krystkowiak P, Azulay JP, Tranchant C, Broussolle E, Morin F, Bachoud-Levi AC, Maison P. Neuroendocrine disturbances in Huntington’s disease. PLoS One. 2009;4(3):e4962; DOI:10.1371/journal.pone.0004962.
  11. Chen JY, Wang EA, Cepeda C, Levine MS. Dopamine imbalance in Hun­tington’s disease: a mechanism for the lack of behavioral flexibility. Front Neurosci. 2013;7:114; DOI:10.3389/fnins.2013.00114.
  12. Xing Y, Lerario AM, Rainey W, Hammer GD. Development of adrenal cortex zonation. Endocrinol Metab Clin North Am. 2015;44(2):243-74; DOI:10.1016/j.ecl.2015.02.001.
  13. Bibb JA, Yan Z, Svenningsson P, Snyder GL, Pieribone VA, Horiuchi A, Na­irn AC, Messer A, Greengard P. Severe deficiencies in dopamine signaling in presymptomatic Huntington’s disease mice. Proc Natl Acad Sci USA. 2000;97(12):6809-14; DOI:10.1073/pnas.120166397.
  14. Cepeda C, Murphy KP, Parent M, Levine MS. The role of dopamine in Huntington’s disease. Prog Brain Res. 2014;211:235-54; DOI:10.1016/B978-0-444-63425-2.00010-6.
  15. Mielcarek M, Landles C, Weiss A, Bradaia A, Seredenina T, Inuabasi L, Osborne GF, Wadel K, Touller C, Butler R, Robertson J, Franklin SA, Smith DL, Park L, Marks PA, Wanker EE, Olson EN, Luthi-Carter R, van der Putten H, Beaumont V, Bates GP. HDAC4 reduction: a novel thera­peutic strategy to target cytoplasmic huntingtin and ameliorate neuro­degeneration. PLoS Biol. 2013;11(11):e1001717; DOI:10.1371/journal. pbio.1001717.
  16. Agustin-Pavon C, Mielcarek M, Garriga-Canut M, Isalan M. Deimmuniza­tion for gene therapy: host matching of synthetic zinc finger constructs enables long-term mutant Huntingtin repression in mice. Mol Neurode­gener. 2016;11(1):64; DOI:10.1186/s13024-016-0128-x.
  17. Kanehisa M, Goto S. KEGG: kyoto encyclopedia of genes and genomes. Nucleic Acids Res. 2000;28(1):27-30; DOI:10.1093/nar/28.1.27.
  18. Kanehisa M. Toward understanding the origin and evolution of cellular organisms. Protein Sci. 2019;28(11):1947-51; DOI:10.1002/pro.3715.
  19. Kanehisa M, Furumichi M, Sato Y, Matsuura Y, Ishiguro-Watanabe M. KEGG: biological systems database as a model of the real world. Nucleic Acids Res. 2025;53(D1):D672-D7; DOI:10.1093/nar/gkae909.
  20. Diaz-Llera S, Podlutsky A, Osterholm AM, Hou SM, Lambert B. Hydro­gen peroxide induced mutations at the HPRT locus in primary hu­man T-lymphocytes. Mutat Res. 2000;469(1):51-61; DOI:10.1016/s1383-5718(00)00058-9.
  21. Vinokurov AY, Soldatov VO, Seregina ES, Dolgikh AI, Tagunov PA, Dunaev AV, Skorkina MY, Deykin AV, Abramov AY. HPRT1 deficiency induces alte­ration of mitochondrial energy metabolism in the brain. Mol Neurobiol. 2023;60(6):3147-57; DOI:10.1007/s12035-023-03266-2.
  22. Jimenez-Jimenez FJ, Alonso-Navarro H, Garcia-Martin E, Carcamo­-Fonfria A, Caballero-Munoz MDM, Agundez JAG. Oxidative stress in Huntington’s disease. Biomolecules. 2025;15(4):527; DOI:10.3390/biom15040527.
  23. Mangiarini L, Sathasivam K, Seller M, Cozens B, Harper A, Hetherington C, Lawton M, Trottier Y, Lehrach H, Davies SW, Bates GP. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell. 1996;87(3):493-506; DOI:10.1016/s0092-8674(00)81369-0.
  24. Du X, Leang L, Mustafa T, Renoir T, Pang TY, Hannan AJ. Environmental enrichment rescues female-specific hyperactivity of the hypothalamic­-pituitary-adrenal axis in a model of Huntington’s disease. Transl Psy­chiatry. 2012;2(7):e133; DOI:10.1038/tp.2012.58.
  25. Bjorkqvist M, Petersen A, Bacos K, Isaacs J, Norlen P, Gil J, Popovic N, Sun­dler F, Bates GP, Tabrizi SJ, Brundin P, Mulder H. Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mo­use model of Huntington’s disease. Hum Mol Genet. 2006;15(10):1713-21; DOI:10.1093/hmg/ddl094.
  26. Spokes EG. Neurochemical alterations in Huntington’s chorea: a study of post-mortem brain tissue. Brain. 1980;103(1):179-210; DOI:10.1093/brain/103.1.179.
  27. Belendiuk K, Belendiuk GW, Freedman DX. Blood monoamine metabo­lism in Huntington’s disease. Arch Gen Psychiatry. 1980;37(3):325-32; DOI:10.1001/archpsyc.1980.01780160095011.
  28. Kalliolia E, Silajdzic E, Nambron R, Costelloe SJ, Martin NG, Hill NR, Frost C, Watt HC, Hindmarsh P, Bjorkqvist M, Warner TT. A 24-hour study of the hypothalamo-pituitary axes in Huntington’s disease. PLoS One. 2015;10(10):e0138848; DOI:10.1371/journal.pone.0138848.
  29. Shirbin CA, Chua P, Churchyard A, Hannan AJ, Lowndes G, Stout JC. The relationship between cortisol and verbal memory in the early stages of Huntington’s disease. J Neurol. 2013;260(3):891-902; DOI:10.1007/s00415-012-6732-y.
Language: English
Page range: 31 - 39
Submitted on: Sep 1, 2025
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Accepted on: Oct 7, 2025
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Published on: Dec 18, 2025
In partnership with: Paradigm Publishing Services
Publication frequency: 4 issues per year

© 2025 Anna Olechnowicz, Małgorzata Blatkiewicz, Mark Isalan, Agnieszka Koc, Michal Mielcarek, Marcin Rucinski, published by Foundation for Cell Biology and Molecular Biology
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.