Type-I protein-C deficiency caused by disruption of a hepatocyte nuclear factor (HNF)-6/HNF-1 binding site in the human protein-C gene promoter

Trends Cardiovasc Med. 1999 Apr-May;9(3-4):82-5. doi: 10.1016/s1050-1738(99)00010-9.

Abstract

The level and tissue specificity of eukaryotic gene transcription is determined by the binding of specific transcription factors to DNA sequence elements located around the transcription start site. The availability and activity of specific transcription factors depends on a variety of developmental and environmental cues and, therefore, varies from cell type to cell type. For instance, liver tissue, the principal site of expression of the coagulation inhibitor protein C, expresses a heterogeneous group of transcription factors called hepatocyte nuclear factors (HNFs). Some of these HNFs are essential players in protein-C gene expression. This review discusses the significance of HNF-1 and HNF-6 in regulating the transcription of the protein-C gene and gives directions for future research.

Publication types

  • Review

MeSH terms

  • DNA / genetics*
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / metabolism*
  • Gene Expression
  • Hepatocyte Nuclear Factor 1
  • Hepatocyte Nuclear Factor 1-alpha
  • Hepatocyte Nuclear Factor 1-beta
  • Hepatocyte Nuclear Factor 6
  • Homeodomain Proteins / genetics
  • Homeodomain Proteins / metabolism*
  • Humans
  • Mutation
  • Nuclear Proteins*
  • Promoter Regions, Genetic*
  • Protein C / genetics
  • Protein C / metabolism*
  • Protein C Deficiency / genetics*
  • Protein C Deficiency / metabolism
  • Trans-Activators / genetics
  • Trans-Activators / metabolism*
  • Transcription Factors / genetics
  • Transcription Factors / metabolism*
  • Transcription, Genetic

Substances

  • DNA-Binding Proteins
  • HNF1A protein, human
  • HNF1B protein, human
  • Hepatocyte Nuclear Factor 1-alpha
  • Hepatocyte Nuclear Factor 6
  • Homeodomain Proteins
  • Nuclear Proteins
  • ONECUT1 protein, human
  • Protein C
  • Trans-Activators
  • Transcription Factors
  • Hepatocyte Nuclear Factor 1
  • Hepatocyte Nuclear Factor 1-beta
  • DNA