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Scientific article
English

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

Published inMolecular and cellular biology, vol. 12, no. 9, p. 4076-4083
Publication date1992
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 Sequence
  • Cell Line, Transformed
  • Cloning, Molecular
  • DNA/ metabolism
  • DNA-Binding Proteins/genetics/ metabolism
  • Gene Expression Regulation
  • Genes, MHC Class II
  • Humans
  • Leukemia, B-Cell/genetics/immunology
  • Molecular Sequence Data
  • Mutation
  • Promoter Regions, Genetic
  • Severe Combined Immunodeficiency/genetics
  • Transcription Factors/genetics/ metabolism
  • Tumor Cells, Cultured
Citation (ISO format)
HERRERO SANCHEZ, Carlos et al. 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. In: Molecular and cellular biology, 1992, vol. 12, n° 9, p. 4076–4083.
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ISSN of the journal0270-7306
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