Summary information and primary citation

PDB-id
2h8r; SNAP-derived features in text and JSON formats; DNAproDB
Class
transcription activator-DNA
Method
X-ray (3.2 Å)
Summary
Hepatocyte nuclear factor 1b bound to DNA: mody5 gene product
Reference
Lu P, Rha GB, Chi YI (2007): "Structural basis of disease-causing mutations in hepatocyte nuclear factor 1beta." Biochemistry, 46, 12071-12080. doi: 10.1021/bi7010527.
Abstract
HNF1beta is an atypical POU transcription factor that participates in a hierarchical network of transcription factors controlling the development and proper function of vital organs such as liver, pancreas, and kidney. Many inheritable mutations on HNF1beta are the monogenic causes of diabetes and several kidney diseases. To elucidate the molecular mechanism of its function and the structural basis of mutations, we have determined the crystal structure of human HNF1beta DNA binding domain in complex with a high-affinity promoter. Disease-causing mutations have been mapped to our structure, and their predicted effects have been tested by a set of biochemical/ functional studies. These findings together with earlier findings with a homologous protein HNF1alpha, help us to understand the structural basis of promoter recognition by these atypical POU transcription factors and the site-specific functional disruption by disease-causing mutations.

Cartoon-block schematics in six views (download the tarball)

PyMOL session file Download PDB file View in 3Dmol.js