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The DNA-binding defect observed in major histocompatibility complex class II regulatory mutants concerns only one member of a family of complexes binding to the X boxes of class II promoters

Silacci, P.
Published in Molecular and Cellular Biology. 1992, vol. 12, no. 9, p. 4076-4083
Abstract The X box of major histocompatibility complex class II promoters is essential for proper expression of class II genes. Here we show that two distinct protein-DNA complexes (A and B), which exhibit similar binding characteristics and identical contact points on the X box, can be formed. This suggests the existence of a family of related X box-binding factors. Complex B (and not complex A) is specifically affected in primary combined immunodeficiency, a congenital defect in class II gene regulation. RFX1, the first X box-binding protein cloned, encodes a functionally relevant factor present in complex A and not in complex B as originally suspected. This report also illustrates the need for caution in correlating specific cloned proteins with nuclear factors identified by DNA-binding assays, particularly when dealing with families of related proteins.
Keywords Base SequenceCell Line, TransformedCloning, MolecularDNA/ metabolismDNA-Binding Proteins/genetics/ metabolismGene Expression RegulationGenes, MHC Class IIHumansLeukemia, B-Cell/genetics/immunologyMolecular Sequence DataMutationPromoter Regions, GeneticSevere Combined Immunodeficiency/geneticsTranscription Factors/genetics/ metabolismTumor Cells, Cultured
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Other version: http://mcb.asm.org/cgi/reprint/12/9/4076.pdf
PMID: 1508204
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