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Immunological observations on patients with Lesch-Nyhan syndrome, and on the role of de-novo purine synthesis in lymphocyte transformation.
- Source :
-
Lancet (London, England) [Lancet] 1975 Dec 13; Vol. 2 (7946), pp. 1179-83. - Publication Year :
- 1975
-
Abstract
- Three patients with the Lesch-Nyhan syndrome were found to have normal delayed hypersensitivity, peripheral-blood T-lymphocyte counts, lymphocyte responses to P.H.A., and serum IgM, IgA, and IgE levels. However, the percentages of B-lymphocytes, IgG levels, serum-isohaemagglutinin titres, and lymphocyte responses to pokeweed mitogen (P.W.M.) were subnormal. These observations suggest that activity of the salvage pathway of purine synthesis catalysed by hypoxanthine-guanine phosphoribosyl transferase (H.G.P.R.T.) is not required for the responses of T-lymphocytes to mitogenic or antigenic stimulation, but may contribute to the proliferation and function of B lymphocytes. The major role of the de-novo pathway of purine synthesis in human lymphocyte responses to mitogenic or antigenic stimulation is shown by the effects of inhibitors of this pathway, including immunosuppressive agents, and by the effects of congenital deficiency or inhibition of adenosine deaminase.
- Subjects :
- Adenosine Deaminase deficiency
Adolescent
Azaserine pharmacology
B-Lymphocytes immunology
Child
Child, Preschool
Hemagglutinins isolation & purification
Humans
Hypersensitivity, Delayed diagnosis
Hypoxanthine Phosphoribosyltransferase deficiency
Immunoglobulins isolation & purification
In Vitro Techniques
Lectins pharmacology
Lesch-Nyhan Syndrome etiology
Lymphocytes drug effects
Male
Mercaptopurine pharmacology
Mitogens immunology
T-Lymphocytes immunology
Thymidine antagonists & inhibitors
Immunologic Deficiency Syndromes complications
Lesch-Nyhan Syndrome immunology
Lymphocyte Activation
Purines biosynthesis
Subjects
Details
- Language :
- English
- ISSN :
- 0140-6736
- Volume :
- 2
- Issue :
- 7946
- Database :
- MEDLINE
- Journal :
- Lancet (London, England)
- Publication Type :
- Academic Journal
- Accession number :
- 53661
- Full Text :
- https://doi.org/10.1016/s0140-6736(75)92661-6