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Molecular and Cellular Biology, June 2000, p. 4455-4461, Vol. 20, No. 12
0270-7306/00/$04.00+0
Copyright © 2000, American Society for Microbiology. All rights reserved.

Mutations in the Bare Lymphocyte Syndrome Define Critical Steps in the Assembly of the Regulatory Factor X Complex

Nada Nekrep, Nabila Jabrane-Ferrat, and B. Matija Peterlin*

Howard Hughes Medical Institute, Departments of Medicine, Microbiology and Immunology, University of California, San Francisco, California 94143-0703

Received 28 December 1999/Returned for modification 3 March 2000/Accepted 13 March 2000

The regulatory factor X (RFX) complex, which contains RFXANK(B), RFXAP, and RFX5, binds to X and S boxes in major histocompatibility complex class II (MHC II) promoters. In the bare lymphocyte syndrome (BLS), which is a human severe combined immunodeficiency, MHC II promoters are neither occupied nor transcribed. Thus, the absence of any one subunit prevents the formation of the RFX complex. Nevertheless, except for a weak binding between RFX5 and RFXAP, no other interactions between RFX proteins have been described. In this study, we demonstrate that RFXANK(B) binds to RFXAP to form a scaffold for the assembly of the RFX complex, which then binds to DNA. Moreover, mutant RFXANK(B) and RFXAP proteins from complementation groups B and D of BLS, respectively, cannot support this interaction. Our data elucidate an intriguing medical situation, where a genetic disease targets two different surfaces that are required for the nucleation of a multisubunit DNA-protein complex.

* Corresponding author. Mailing address: Room N215, UCSF Mt. Zion Cancer Center, 2340 Sutter St., San Francisco, CA 94115. Phone: (415) 502-1902. Fax: (415) 502-1901. E-mail: matija{at}itsa.ucsf.edu.

Molecular and Cellular Biology, June 2000, p. 4455-4461, Vol. 20, No. 12
0270-7306/00/$04.00+0
Copyright © 2000, American Society for Microbiology. All rights reserved.


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