The DOI matches its Crossref record; 24 of the 24 references it cites were found in a registry or an index.
A novel lymphoproliferative/autoimmune syndrome resembling murine lpr/gld disease.
Open accessAbstract
In mice, the two distinct autosomal recessive genes lpr and gld can induce a syndrome characterized by autoantibody formation and the progressive accumulation of an unusual CD4-CD8- T cell population in peripheral lymphoid tissue. This phenotype does not precisely mirror any human disease. In this report we describe two patients with a progressive lymphoproliferative disorder associated with autoimmunity. The peripheral blood and lymph nodes of these patients contained large numbers of an unusual CD4-CD8- T cell population. These CD4-CD8- T cells express surface markers characteristic of mature peripheral blood T cells (CD3, CD2, CD5), express the alpha/beta form of the T cell receptor, and do not express surface markers characteristic of immature thymocytes (CD1) or NK cells (CD16, CD56). Functionally, these cells exhibited deficient proliferation and lymphokine production upon stimulation with mitogenic antibodies to CD3 or CD2. Both proliferation and lymphokine production could be augmented by co-stimulation with an antibody directed at the CD28 determinant. The clinical and immunological features of this syndrome resemble the lymphoproliferative/autoimmune disease seen in lpr and gld mice.
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The multifaceted role of Fas signaling in immune cell homeostasis and autoimmunity
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Metadata
- Authors
- Sneller, M. C., Straus, S. E., Jaffe, E. S., Jaffe, J. S., Fleisher, T. A., Stetler-Stevenson, M., & Strober, W.
- Venue
- Journal of Clinical Investigation
- Published
- 1992-08-01
- Volume
- 90
- Issue
- 2
- Pages
- 334-341
- ISSN
- 0021-9738 (ISSN-L)
- OpenAlex
- W2137950352
- Cited by
- 301
- References
- 24
- FWCI
- 2.48
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Sneller, M. C., Straus, S. E., Jaffe, E. S., Jaffe, J. S., Fleisher, T. A., Stetler-Stevenson, M., & Strober, W. (1992). A novel lymphoproliferative/autoimmune syndrome resembling murine lpr/gld disease. Journal of Clinical Investigation. https://doi.org/10.1172/jci115867
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