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. 1984 Jul;74(1):165–172. doi: 10.1172/JCI111397

Hypersensitivity to antigen derived from brain tissue in patients with Huntington's disease and in individuals at risk.

R S Walls, A Ruwoldt
PMCID: PMC425197  PMID: 6234326

Abstract

The hypothesis that an immune response is directed to components of brain tissue in Huntington's disease (HD) was examined. 27 patients with HD, 21 children of affected parents who showed no signs of disease, 6 spouses of HD patients, and 40 patients with other neurologic diseases were investigated. Leukocyte migration inhibition to antigens prepared from brain tissue was demonstrated in 26 HD patients and in 9 children. None of the six spouses reacted. 13 patients with other neurologic conditions (including 60% of patients after cerebrovascular accidents) reacted to rat brain extract. Fractionation of brain tissue indicated that the material responsible for reactions in these patients differed from that in HD. The material that elicited hypersensitivity in HD was not species specific, since it was found in rat, calf, human, and rabbit brain. It was not present in rat kidney, liver, or heart. Preliminary characterization showed that the material was protein in nature, contained sugar and lipid, and was heat labile. The finding of hypersensitivity in about half the unaffected children of HD parents suggests that it may precede the clinical onset of disease and serve as a marker for the gene.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Barkley D. S., Hardiwidjaja S. I., Menkes J. H., Ellison G. W., Myers L. W. Cellular immune responses in Huntington's disease (H.D.). Detection of H.D. and multiple sclerosis (M.S.) brain antigenicity by H.D. but not M.S. lymphocytes. Cell Immunol. 1977 Aug;32(2):385–390. doi: 10.1016/0008-8749(77)90214-3. [DOI] [PubMed] [Google Scholar]
  2. Becker P. E. Genetic approaches to the nosology of muscular disease: myotonias and similar diseases. Birth Defects Orig Artic Ser. 1971 Feb;7(2):52–62. [PubMed] [Google Scholar]
  3. Gusella J. F., Wexler N. S., Conneally P. M., Naylor S. L., Anderson M. A., Tanzi R. E., Watkins P. C., Ottina K., Wallace M. R., Sakaguchi A. Y. A polymorphic DNA marker genetically linked to Huntington's disease. Nature. 1983 Nov 17;306(5940):234–238. doi: 10.1038/306234a0. [DOI] [PubMed] [Google Scholar]
  4. Hardiwidjaja S. I., Morgan A., Ellison G. W., Myers L. W., Pierce W. G., Barkley D. S. Huntington's disease and leucocyte adherence inhibition. (LAI). Clin Exp Immunol. 1981 Sep;45(3):583–589. [PMC free article] [PubMed] [Google Scholar]
  5. Husby G., Li L., Davis L. E., Wedege E., Kokmen E., Williams R. C., Jr Antibodies to human caudate nucleus neurons in Huntington's chorea. J Clin Invest. 1977 May;59(5):922–932. doi: 10.1172/JCI108714. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Husby G., Wedege E., Williams R. C., Jr Characterization of brain proteins reacting in vitro with anti-neuronal antibodies in patients with Huntington's disease. Clin Immunol Immunopathol. 1978 Oct;11(2):131–141. doi: 10.1016/0090-1229(78)90038-7. [DOI] [PubMed] [Google Scholar]
  7. Johnson C., Walls R. S., Ruwoldt A. Delayed hypersensitivity to tetanus toxoid in man: in vivo and in vitro studies. Pathology. 1983 Oct;15(4):369–372. doi: 10.3109/00313028309085161. [DOI] [PubMed] [Google Scholar]
  8. LOWRY O. H., ROSEBROUGH N. J., FARR A. L., RANDALL R. J. Protein measurement with the Folin phenol reagent. J Biol Chem. 1951 Nov;193(1):265–275. [PubMed] [Google Scholar]
  9. Martin J. B. Huntington's disease: genetically programmed cell death in the human central nervous system. Nature. 1982 Sep 16;299(5880):205–206. doi: 10.1038/299205a0. [DOI] [PubMed] [Google Scholar]
  10. Michetti F., Massaro A., Russo G., Rigon G. The S-100 antigen in cerebrospinal fluid as a possible index of cell injury in the nervous system. J Neurol Sci. 1980 Jan;44(2-3):259–263. doi: 10.1016/0022-510x(80)90133-1. [DOI] [PubMed] [Google Scholar]
  11. Mitchell C. G., Smith M. G., Golding P. L., Eddleston A. L., Williams R. Evaluation of the leucocyte migration test as a measure of delayed hypersensitivty in man. Suppression of migration inhibition by puromycin. Clin Exp Immunol. 1972 Aug;11(4):535–541. [PMC free article] [PubMed] [Google Scholar]
  12. Soborg M., Bendixen G. Human lymphocyte migration as a parameter of hypersensitivity. Acta Med Scand. 1967 Feb;181(2):247–256. doi: 10.1111/j.0954-6820.1967.tb07255.x. [DOI] [PubMed] [Google Scholar]

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