| http://purl.uniprot.org/citations/15647252 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/15647252 | http://www.w3.org/2000/01/rdf-schema#comment | "Hepatocyte nuclear factor-1beta (HNF-1beta) is a homeodomain-containing transcription factor that regulates tissue-specific gene expression in the kidney and other epithelial organs. Mutations of HNF-1beta produce congenital cystic abnormalities of the kidney, and previous studies showed that HNF-1beta regulates the expression of the autosomal recessive polycystic kidney disease (ARPKD) gene, Pkhd1. Here we show that the C-terminal region of HNF-1beta contains an activation domain that is functional when fused to a heterologous DNA-binding domain. An HNF-1beta deletion mutant lacking the C-terminal domain interacts with wild-type HNF-1beta, binds DNA, and functions as a dominant-negative inhibitor of a chromosomally integrated Pkhd1 promoter. The activation of the Pkhd1 promoter by wild-type HNF-1beta is stimulated by sodium butyrate or coactivators CREB (cAMP-response element)-binding protein (CBP) and P/CAF. The interaction with CBP and P/CAF requires the C-terminal domain. Expression of an HNF-1beta C-terminal deletion mutant in transgenic mice produces renal cysts, increased cell proliferation, and dilatation of the ureter similar to mice with kidney-specific inactivation of HNF-1beta. Pkhd1 expression is inhibited in cystic collecting ducts but not in non-cystic proximal tubules, despite transgene expression in this nephron segment. We conclude that the C-terminal domain of HNF-1beta is required for the activation of the Pkhd1 promoter. Deletion mutants lacking the C-terminal domain function as dominant-negative mutants, possibly by preventing the recruitment of histone acetylases to the promoter. Cyst formation correlates with inhibition of Pkhd1 expression, which argues that mutations of HNF-1beta produce kidney cysts by down-regulating the ARPKD gene, Pkhd1. Expression of HNF-1alpha in proximal tubules may protect against cystogenesis."xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.m414121200"xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/author | "Shao X."xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/author | "Igarashi P."xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/author | "Pontoglio M."xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/author | "Reimann A."xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/author | "Hiesberger T."xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/author | "Gourley E."xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/date | "2005"xsd:gYear |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/name | "J Biol Chem"xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/pages | "10578-10586"xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/title | "Role of the hepatocyte nuclear factor-1beta (HNF-1beta) C-terminal domain in Pkhd1 (ARPKD) gene transcription and renal cystogenesis."xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://purl.uniprot.org/core/volume | "280"xsd:string |
| http://purl.uniprot.org/citations/15647252 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/15647252 |
| http://purl.uniprot.org/citations/15647252 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/15647252 |
| http://purl.uniprot.org/uniprot/Q9JHD1#attribution-3D03B2365E779D83851C0AA799EA4D50 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/15647252 |
| http://purl.uniprot.org/uniprot/P27889#attribution-3D03B2365E779D83851C0AA799EA4D50 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/15647252 |
| http://purl.uniprot.org/uniprot/P27889#attribution-642BCDE9EB13593CD7A3C9A37BD75885 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/15647252 |
| http://purl.uniprot.org/uniprot/P27889#attribution-B34FBD6CFB7E02044CFD3D869FCB3CA5 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/15647252 |
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