Article
Mutations in the bare lymphocyte syndrome define critical steps in the assembly of the regulatory factor X complex.
Molecular and cellular biology - 1 Jun 2000
Nekrep N, Jabrane-Ferrat N, Peterlin B M
Abstract excerpt
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...
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