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Updated: Jul 24, 2025

Assembly of Nucleosomal Arrays from Recombinant Core Histones and Nucleosome Positioning DNA
Published on: September 10, 2013
Structural insights into the HNF4 biology
Brice Beinsteiner1,2,3,4, Isabelle M L Billas1,2,3,4, Dino Moras1,2,3,4
1Laboratory IGBMC (Institute of Genetics and of Molecular and Cellular Biology), Centre for Integrative Biology (CBI), Illkirch, France.
Hepatocyte Nuclear Factor 4 (HNF4) is a key transcription factor regulating liver genes. This review examines HNF4 structures, disease links, and how mutations and modifications impact its function.
Area of Science:
- Molecular Biology
- Structural Biology
- Genetics
Background:
- Hepatocyte Nuclear Factor 4 (HNF4) is a crucial transcription factor (TF) in the nuclear receptor (NR) family.
- It plays a vital role in liver-specific gene expression, particularly for lipid and glucose metabolism, and is essential for development.
- HNF4 dysregulation is implicated in diseases like type I diabetes (MODY1) and hemophilia.
Purpose of the Study:
- To review the structures of the DNA binding domain (DBD) and ligand binding domain (LBD) of HNF4.
- To compare HNF4 structures with other nuclear receptors.
- To discuss HNF4α biology from a structural viewpoint, focusing on mutations and post-translational modifications.
Main Methods:
- Structural analysis of isolated HNF4 DNA binding domain (DBD) and ligand binding domain (LBD).
- Comparison of HNF4 structures with other nuclear receptors.
- Review of existing literature on HNF4α mutations and post-translational modifications.
Main Results:
- Detailed comparison of HNF4 DBD and LBD structures with other NRs.
- Insights into how pathological mutations affect HNF4 structure and function.
- Understanding the impact of post-translational modifications on HNF4α receptor activity.
Conclusions:
- Structural insights into HNF4 are critical for understanding its regulatory roles.
- Pathological mutations and post-translational modifications significantly influence HNF4 structure-function relationships.
- This review provides a structural perspective on HNF4 biology and its disease relevance.
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