| http://purl.uniprot.org/citations/20933420 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/20933420 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/20933420 | http://www.w3.org/2000/01/rdf-schema#comment | "Transport of chloride through the cystic fibrosis transmembrane conductance regulator (CFTR) channel is a key step in regulating fluid secretion in vertebrates [1, 2]. Loss of CFTR function leads to cystic fibrosis [1, 3, 4], a disease that affects the lungs, pancreas, liver, intestine, and vas deferens. Conversely, uncontrolled activation of the channel leads to increased fluid secretion and plays a major role in several diseases and conditions including cholera [5, 6] and other secretory diarrheas [7] as well as polycystic kidney disease [8-10]. Understanding how CFTR activity is regulated in vivo has been limited by the lack of a genetic model. Here, we used a forward genetic approach in zebrafish to uncover CFTR regulators. We report the identification, isolation, and characterization of a mutation in the zebrafish cse1l gene that leads to the sudden and dramatic expansion of the gut tube. We show that this phenotype results from a rapid accumulation of fluid due to the uncontrolled activation of the CFTR channel. Analyses in zebrafish larvae and mammalian cells indicate that Cse1l is a negative regulator of CFTR-dependent fluid secretion. This work demonstrates the importance of fluid homeostasis in development and establishes the zebrafish as a much-needed model system to study CFTR regulation in vivo."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.cub.2010.09.012"xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.cub.2010.09.012"xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Brand-Arzamendi K."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Brand-Arzamendi K."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Stainier D.Y."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Stainier D.Y."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Bagnat M."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Bagnat M."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Gabriel S."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Gabriel S."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Curado S."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Curado S."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Herbstreith S."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Herbstreith S."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Huisken J."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Huisken J."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Mostov K."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Mostov K."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Navis A."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/author | "Navis A."xsd:string |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/date | "2010"xsd:gYear |
| http://purl.uniprot.org/citations/20933420 | http://purl.uniprot.org/core/date | "2010"xsd:gYear |