Abstract
HL-A phenotypes were determined by a cytotoxicity assay on circulating lymphocytes of two boys with the premature aging syndrome, progeria. The antigenic phenotypes were not unusual inasmuch as they are frequently seen in the normal population. However, none of these antigens could be detected by the same assay on cultured skin fibroblasts from either individual, even when a significant mitotic potential remained before cessation of growth. Fibroblasts from normal donors were concordant with corresponding lymphocytes for HL-A antigens and maintained these antigens until mitotic division had virtually ceased. Absorption studies on fibroblasts with two HL-A2 antisera revealed that HL-A antigens are either absent or have a drastically reduced expression on progeric fibroblasts. The data are in accord with the concept of an immunological role in the pathogenesis of progeria.
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Selected References
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- Amos D. B. Genetic and antigenetic aspects of human histocompatibility systems. Adv Immunol. 1969;10:251–297. doi: 10.1016/s0065-2776(08)60419-8. [DOI] [PubMed] [Google Scholar]
- Brautbar C., Payne R., Hayflick L. Fate of HL-A antigens in aging cultured human diploid cell strains. Exp Cell Res. 1972 Nov;75(1):31–38. doi: 10.1016/0014-4827(72)90516-2. [DOI] [PubMed] [Google Scholar]
- Danes B. S. Progeria: a cell culture study on aging. J Clin Invest. 1971 Sep;50(9):2000–2003. doi: 10.1172/JCI106692. [DOI] [PMC free article] [PubMed] [Google Scholar]
- DeBusk F. L. The Hutchinson-Gilford progeria syndrome. Report of 4 cases and review of the literature. J Pediatr. 1972 Apr;80(4):697–724. doi: 10.1016/s0022-3476(72)80229-4. [DOI] [PubMed] [Google Scholar]
- Epstein J., Williams J. R., Little J. B. Deficient DNA repair in human progeroid cells. Proc Natl Acad Sci U S A. 1973 Apr;70(4):977–981. doi: 10.1073/pnas.70.4.977. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Gershon H., Gershon D. Inactive enzyme molecules in aging mice: liver aldolase. Proc Natl Acad Sci U S A. 1973 Mar;70(3):909–913. doi: 10.1073/pnas.70.3.909. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Goldstein S. Analytical review: the pathogenesis of diabetes mellitus and its relationship to biological aging. Humangenetik. 1971;12(2):83–100. doi: 10.1007/BF00291463. [DOI] [PubMed] [Google Scholar]
- Goldstein S. Lifespan of cultured cells in progeria. Lancet. 1969 Feb 22;1(7591):424–424. doi: 10.1016/s0140-6736(69)91404-4. [DOI] [PubMed] [Google Scholar]
- Goldstein S., Littlefield J. W., Soeldner J. S. Diabetes mellitus and aging: diminished planting efficiency of cultured human fibroblasts. Proc Natl Acad Sci U S A. 1969 Sep;64(1):155–160. doi: 10.1073/pnas.64.1.155. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Goldstein S., Singal D. P. Loss of reactivity of HL-A antigens in clonal populations of cultured human fibroblasts durin aging in vitro. Exp Cell Res. 1972 Nov;75(1):278–282. doi: 10.1016/0014-4827(72)90549-6. [DOI] [PubMed] [Google Scholar]
- Goldstein S. The biology of aging. N Engl J Med. 1971 Nov;285(20):1120–1129. doi: 10.1056/NEJM197111112852005. [DOI] [PubMed] [Google Scholar]
- HAYFLICK L. THE LIMITED IN VITRO LIFETIME OF HUMAN DIPLOID CELL STRAINS. Exp Cell Res. 1965 Mar;37:614–636. doi: 10.1016/0014-4827(65)90211-9. [DOI] [PubMed] [Google Scholar]
- Klein G., Friberg S., Jr, Harris H. Two kinds of antigen suppression in tumor cells revealed by cell fusion. J Exp Med. 1972 Apr 1;135(4):839–849. doi: 10.1084/jem.135.4.839. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Lewis C. M., Tarrant G. M. Error theory and ageing in human diploid fibroblasts. Nature. 1972 Oct 6;239(5371):316–318. doi: 10.1038/239316a0. [DOI] [PubMed] [Google Scholar]
- Martin G. M., Sprague C. A., Epstein C. J. Replicative life-span of cultivated human cells. Effects of donor's age, tissue, and genotype. Lab Invest. 1970 Jul;23(1):86–92. [PubMed] [Google Scholar]
- Mittal K. K., Mickey M. R., Singal D. P., Terasaki P. I. Serotyping for homotransplantation. 18. Refinement of microdroplet lymphocyte cytotoxicity test. Transplantation. 1968 Nov;6(8):913–927. doi: 10.1097/00007890-196811000-00006. [DOI] [PubMed] [Google Scholar]
- Reichel W., Garcia-Bunuel R. Pathologic findings in progeria: myocardial fibrosis and lipofuscin pigment. Am J Clin Pathol. 1970 Feb;53(2):243–253. doi: 10.1093/ajcp/53.2.243. [DOI] [PubMed] [Google Scholar]
- Sasportes M., Dehay C., Fellous M. Variations of the expression of HL-A antigens on human diploid fibroblasts in vitro. Nature. 1971 Oct 1;233(5318):332–334. doi: 10.1038/233332a0. [DOI] [PubMed] [Google Scholar]
- Thorsby E., Lie S. Antigens on human fibroblasts demonstrated with HL-A antisera and anti-human lymphocytic sera. Vox Sang. 1968;15(1):44–53. doi: 10.1111/j.1423-0410.1968.tb04446.x. [DOI] [PubMed] [Google Scholar]
- Villee D. B., Nichols G., Jr, Talbot N. B. Metabolic studies in two boys with classical progeria. Pediatrics. 1969 Feb;43(2):207–216. [PubMed] [Google Scholar]
- Walford R. L. Antibody diversity, histocompatibility systems, disease states, and ageing. Lancet. 1970 Dec 12;2(7685):1226–1229. doi: 10.1016/s0140-6736(70)92184-7. [DOI] [PubMed] [Google Scholar]
