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PDBsum entry 2h8r
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Transcription activator/DNA
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PDB id
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2h8r
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References listed in PDB file
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Key reference
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Title
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Structural basis of disease-Causing mutations in hepatocyte nuclear factor 1beta.
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Authors
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P.Lu,
G.B.Rha,
Y.I.Chi.
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Ref.
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Biochemistry, 2007,
46,
12071-12080.
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PubMed id
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Abstract
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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.
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Secondary reference #1
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Title
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Crystallization of hepatocyte nuclear factor 1beta in complex with DNA.
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Authors
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P.Lu,
Y.Li,
A.Gorman,
Y.I.Chi.
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Ref.
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Acta Crystallogr Sect F Struct Biol Cryst Commun, 2006,
62,
525-529.
[DOI no: ]
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PubMed id
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Figure 2.
Overhang 21-mer used to obtain diffraction-quality crystals.
The HNF1[beta]-recognition motif is underlined. Acta Crystallogr
Sect F Struct Biol Cryst Commun. 2006 June 1; 62(Pt 6):
525–529. Published online 2006 May 5. doi:
10.1107/S1744309106015168. Copyright [copyright] International
Union of Crystallography 2006
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Figure 5.
Typical crystals of HNF1[beta] --DNA complex. Approximate
size is indicated by a black bar. Acta Crystallogr Sect F Struct
Biol Cryst Commun. 2006 June 1; 62(Pt 6): 525–529. Published
online 2006 May 5. doi: 10.1107/S1744309106015168. Copyright
[copyright] International Union of Crystallography 2006
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The above figures are
reproduced from the cited reference
which is an Open Access publication published by the IUCr
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