On the structural organization of the intracellular domains of CFTR
The cystic fibrosis transmembrane conductance regulator (CFTR) is a multidomain membrane protein forming an anion selective channel. Mutations in the gene encoding CFTR cause cystic fibrosis (CF). The intracellular side of CFTR constitutes about 80% of the total mass of the protein. This region incl...
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Veröffentlicht in: | The international journal of biochemistry & cell biology 2014-07, Vol.52, p.7-14 |
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description | The cystic fibrosis transmembrane conductance regulator (CFTR) is a multidomain membrane protein forming an anion selective channel. Mutations in the gene encoding CFTR cause cystic fibrosis (CF). The intracellular side of CFTR constitutes about 80% of the total mass of the protein. This region includes domains involved in ATP-dependent gating and regulatory protein kinase-A phosphorylation sites. The high-resolution molecular structure of CFTR has not yet been solved. However, a range of lower resolution structural data, as well as functional biochemical and electrophysiological data, are now available. This information has enabled the proposition of a working model for the structural architecture of the intracellular domains of the CFTR protein.
This article is part of a Directed Issue entitled: Cystic Fibrosis: From o-mics to cell biology, physiology, and therapeutic advances. |
doi_str_mv | 10.1016/j.biocel.2014.01.024 |
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This article is part of a Directed Issue entitled: Cystic Fibrosis: From o-mics to cell biology, physiology, and therapeutic advances.</description><subject>Biochemistry</subject><subject>Biology</subject><subject>CFTR</subject><subject>Channels</subject><subject>Cystic fibrosis</subject><subject>Cystic Fibrosis - genetics</subject><subject>Cystic Fibrosis - metabolism</subject><subject>Cystic Fibrosis Transmembrane Conductance Regulator - chemistry</subject><subject>Cystic Fibrosis Transmembrane Conductance Regulator - genetics</subject><subject>Cystic Fibrosis Transmembrane Conductance Regulator - metabolism</subject><subject>Encoding</subject><subject>Humans</subject><subject>Models, Molecular</subject><subject>Molecular structure</subject><subject>NBD</subject><subject>Phosphorylation</subject><subject>Physiology</subject><subject>Protein Binding</subject><subject>Protein Structure, Tertiary</subject><subject>Proteins</subject><subject>Regulatory domain</subject><subject>Structure</subject><issn>1357-2725</issn><issn>1878-5875</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkNFKwzAUhoMobk7fQKSX3rTmJE3T3ggynAqDgew-ZOmZZrTNTFpBn97WTi_1KiH_d_IfPkIugSZAIbvZJRvrDFYJo5AmFBLK0iMyhVzmscilOO7vXMiYSSYm5CyEHaUUBOOnZMJS0WccpmS-aqL2FaPQ-s60nddV5PyLbuynbq1rIrf9jm3Tet2XVV2lfVS6WtsmDOF8sX4-JydbXQW8OJwzsl7cr-eP8XL18DS_W8aGA21jkwpmCiZA802elsA4zw1DoEZmrBA5gwJRlogG5CbrX1lm8qIwmpYoNeUzcj1-u_furcPQqtqGYSfdoOuCgkyCKDhkxf-oSBkrJMsHNB1R410IHrdq722t_YcCqgbRaqdG0WoQrSioXnQ_dnVo6DY1lr9DP2Z74HYEsDfybtGrYCw2Bkvr0bSqdPbvhi85Ro8K</recordid><startdate>201407</startdate><enddate>201407</enddate><creator>Moran, Oscar</creator><general>Elsevier Ltd</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7U5</scope><scope>8FD</scope><scope>L7M</scope></search><sort><creationdate>201407</creationdate><title>On the structural organization of the intracellular domains of CFTR</title><author>Moran, Oscar</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c310t-c452c9251a3b84d12338c2e10c762958219ee7deec17b60c726c899ca0de7a03</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Biochemistry</topic><topic>Biology</topic><topic>CFTR</topic><topic>Channels</topic><topic>Cystic fibrosis</topic><topic>Cystic Fibrosis - genetics</topic><topic>Cystic Fibrosis - metabolism</topic><topic>Cystic Fibrosis Transmembrane Conductance Regulator - chemistry</topic><topic>Cystic Fibrosis Transmembrane Conductance Regulator - genetics</topic><topic>Cystic Fibrosis Transmembrane Conductance Regulator - metabolism</topic><topic>Encoding</topic><topic>Humans</topic><topic>Models, Molecular</topic><topic>Molecular structure</topic><topic>NBD</topic><topic>Phosphorylation</topic><topic>Physiology</topic><topic>Protein Binding</topic><topic>Protein Structure, Tertiary</topic><topic>Proteins</topic><topic>Regulatory domain</topic><topic>Structure</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Moran, Oscar</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Solid State and Superconductivity Abstracts</collection><collection>Technology Research Database</collection><collection>Advanced Technologies Database with Aerospace</collection><jtitle>The international journal of biochemistry & cell biology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Moran, Oscar</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>On the structural organization of the intracellular domains of CFTR</atitle><jtitle>The international journal of biochemistry & cell biology</jtitle><addtitle>Int J Biochem Cell Biol</addtitle><date>2014-07</date><risdate>2014</risdate><volume>52</volume><spage>7</spage><epage>14</epage><pages>7-14</pages><issn>1357-2725</issn><eissn>1878-5875</eissn><abstract>The cystic fibrosis transmembrane conductance regulator (CFTR) is a multidomain membrane protein forming an anion selective channel. 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subjects | Biochemistry Biology CFTR Channels Cystic fibrosis Cystic Fibrosis - genetics Cystic Fibrosis - metabolism Cystic Fibrosis Transmembrane Conductance Regulator - chemistry Cystic Fibrosis Transmembrane Conductance Regulator - genetics Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Encoding Humans Models, Molecular Molecular structure NBD Phosphorylation Physiology Protein Binding Protein Structure, Tertiary Proteins Regulatory domain Structure |
title | On the structural organization of the intracellular domains of CFTR |
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