The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.
The Bare Lymphocyte Syndrome and the Regulation of MHC Expression
Open accessAbstract
The bare lymphocyte syndrome (BLS) is a hereditary immunodeficiency resulting from the absence of major istocompatibility complex class II (MHCII) expression. Considering the central role of MHCII molecules in the development and activation of CD4+T cells, it is not surprising that the immune system of the patients is severely impaired. BLS is the prototype of a “disease of gene regulation.” The affected genes encode RFXANK, RFX5, RFXAP, and CIITA, four regulatory factors that are highly specific and essential for MHCII genes. The first three are subunits of RFX, a trimeric complex that binds to all MHCII promoters. CIITA is a non-DNA-binding coactivator that functions as the master control factor for MHCII expression. The study of RFX and CIITA has made major contributions to our comprehension of the molecular mechanisms controlling MHCII genes and has made this system into a textbook model for the regulation of gene expression.
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Cites
Regulation of MHC Class II Expression by Interferon-γ Mediated by the Transactivator Gene CIITA
Steimle, V., Siegrist, C.-A., Mottet, A., Lisowska-Grospierre, B., & Mach, B. (1994) · Science · doi:10.1126/science.8016643 · Cited by 898
Nod1, an Apaf-1-like Activator of Caspase-9 and Nuclear Factor-κB
Inohara, N., Koseki, T., del Peso, L., Hu, Y., Yee, C., Chen, S., Carrio, R., Merino, J., Liu, D., Ni, J., et al. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.21.14560 · Cited by 742
Imada, K., & Leonard, W. J. (2000) · Molecular Immunology · doi:10.1016/s0161-5890(00)00018-3 · Cited by 610
Induction of MHC Class I Expression by the MHC Class II Transactivator CIITA
Martin, B. K., Chin, K.-C., Olsen, J. C., Skinner, C. A., Dey, A., Ozato, K., & Ting, J. P.-Y. (1997) · Immunity · doi:10.1016/s1074-7613(00)80347-7 · Cited by 208
Cited by
The Inflammasomes: Guardians of the Body
Martinon, F., Mayor, A., & Tschopp, J. (2009) · Annual Review of Immunology · doi:10.1146/annurev.immunol.021908.132715 · Cited by 2,436
NOD-LRR PROTEINS: Role in Host-Microbial Interactions and Inflammatory Disease
Inohara, N., Chamaillard, M., McDonald, C., & Nuñez, G. (2005) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.74.082803.133347 · Cited by 991
The NLR Gene Family: A Standard Nomenclature
Ting, J. P.-Y., Lovering, R. C., Alnemri, E. S., Bertin, J., Boss, J. M., Davis, B. K., Flavell, R. A., Girardin, S. E., Godzik, A., Harton, J. A., et al. (2008) · Immunity · doi:10.1016/j.immuni.2008.02.005 · Cited by 955
Self-citationNod-like proteins in immunity, inflammation and disease
Fritz, J. H., Ferrero, R. L., Philpott, D. J., & Girardin, S. E. (2006) · Nature Immunology · doi:10.1038/ni1412 · Cited by 892
NOD-Like Receptors: Role in Innate Immunity and Inflammatory Disease
Chen, G., Shaw, M. H., Kim, Y.-G., & Nuñez, G. (2009) · Annual Review of Pathology: Mechanisms of Disease · doi:10.1146/annurev.pathol.4.110807.092239 · Cited by 772
Metadata
- Authors
- Reith, W., & Mach, B.
- Venue
- Annual Review of Immunology
- Publisher
- Annual Reviews
- Published
- 2001-04
- Volume
- 19
- Issue
- 1
- Pages
- 331-373
- ISSN
- 0732-0582 (ISSN-L)
- 1545-3278
- OpenAlex
- W1920211008
- Cited by
- 518
- References
- 4
- FWCI
- 1.82
- Type
- journal article
- Language
- en
- Open access
- green
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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