Paracellin-1, a renal tight junction protein required for paracellular Mg2+ resorption
Epithelia permit selective and regulated flux from apical to basolateral surfaces by transcellular passage through cells or paracellular flux between cells. Tight junctions constitute the barrier to paracellular conductance; however, little is known about the specific molecules that mediate paracell...
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| Veröffentlicht in: | Science (American Association for the Advancement of Science) 1999-07, Vol.285 (5424), p.103-106 |
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| creator | SIMON, D. B YIN LU MCCREDIE, D MILFORD, D SANJAD, S LIFTON, R. P CHOATE, K. A VELAZQUEZ, H AL-SABBAN, E PRAGA, M CASARI, G BETTINELLI, A COLUSSI, G RODRIGUEZ-SORIANO, J |
| description | Epithelia permit selective and regulated flux from apical to basolateral surfaces by transcellular passage through cells or paracellular flux between cells. Tight junctions constitute the barrier to paracellular conductance; however, little is known about the specific molecules that mediate paracellular permeabilities. Renal magnesium ion (Mg2+) resorption occurs predominantly through a paracellular conductance in the thick ascending limb of Henle (TAL). Here, positional cloning has identified a human gene, paracellin-1 (PCLN-1), mutations in which cause renal Mg2+ wasting. PCLN-1 is located in tight junctions of the TAL and is related to the claudin family of tight junction proteins. These findings provide insight into Mg2+ homeostasis, demonstrate the role of a tight junction protein in human disease, and identify an essential component of a selective paracellular conductance. |
| doi_str_mv | 10.1126/science.285.5424.103 |
| format | Article |
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B ; YIN LU ; MCCREDIE, D ; MILFORD, D ; SANJAD, S ; LIFTON, R. P ; CHOATE, K. A ; VELAZQUEZ, H ; AL-SABBAN, E ; PRAGA, M ; CASARI, G ; BETTINELLI, A ; COLUSSI, G ; RODRIGUEZ-SORIANO, J</creator><creatorcontrib>SIMON, D. B ; YIN LU ; MCCREDIE, D ; MILFORD, D ; SANJAD, S ; LIFTON, R. P ; CHOATE, K. A ; VELAZQUEZ, H ; AL-SABBAN, E ; PRAGA, M ; CASARI, G ; BETTINELLI, A ; COLUSSI, G ; RODRIGUEZ-SORIANO, J</creatorcontrib><description>Epithelia permit selective and regulated flux from apical to basolateral surfaces by transcellular passage through cells or paracellular flux between cells. Tight junctions constitute the barrier to paracellular conductance; however, little is known about the specific molecules that mediate paracellular permeabilities. Renal magnesium ion (Mg2+) resorption occurs predominantly through a paracellular conductance in the thick ascending limb of Henle (TAL). Here, positional cloning has identified a human gene, paracellin-1 (PCLN-1), mutations in which cause renal Mg2+ wasting. PCLN-1 is located in tight junctions of the TAL and is related to the claudin family of tight junction proteins. These findings provide insight into Mg2+ homeostasis, demonstrate the role of a tight junction protein in human disease, and identify an essential component of a selective paracellular conductance.</description><identifier>ISSN: 0036-8075</identifier><identifier>EISSN: 1095-9203</identifier><identifier>DOI: 10.1126/science.285.5424.103</identifier><identifier>PMID: 10390358</identifier><identifier>CODEN: SCIEAS</identifier><language>eng</language><publisher>Washington, DC: American Association for the Advancement of Science</publisher><subject>Amino Acid Sequence ; Biological and medical sciences ; Calcium - urine ; Cell physiology ; Cellular biology ; Chromosomes, Human, Pair 3 - genetics ; Claudins ; Cloning ; Cloning, Molecular ; Coding ; Female ; Fundamental and applied biological sciences. Psychology ; Genes ; Genes, Recessive ; Homeostasis ; Humans ; Kidney Diseases - genetics ; Kidney Diseases - metabolism ; Kidney Tubules - chemistry ; Loop of Henle - chemistry ; Loop of Henle - metabolism ; Magnesium - blood ; Magnesium - metabolism ; Magnesium Deficiency - genetics ; Magnesium Deficiency - metabolism ; Male ; Membrane and intracellular transports ; Membrane Proteins - analysis ; Membrane Proteins - chemistry ; Membrane Proteins - genetics ; Membrane Proteins - physiology ; Molecular and cellular biology ; Molecular Sequence Data ; Mutation ; Patients ; Pedigree ; Physical Chromosome Mapping ; Proteins ; Tight Junctions - metabolism ; Vertebrates: urinary system</subject><ispartof>Science (American Association for the Advancement of Science), 1999-07, Vol.285 (5424), p.103-106</ispartof><rights>1999 INIST-CNRS</rights><rights>Copyright American Association for the Advancement of Science Jul 2, 1999</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,782,786,27931,27932</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1866861$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10390358$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>SIMON, D. B</creatorcontrib><creatorcontrib>YIN LU</creatorcontrib><creatorcontrib>MCCREDIE, D</creatorcontrib><creatorcontrib>MILFORD, D</creatorcontrib><creatorcontrib>SANJAD, S</creatorcontrib><creatorcontrib>LIFTON, R. P</creatorcontrib><creatorcontrib>CHOATE, K. A</creatorcontrib><creatorcontrib>VELAZQUEZ, H</creatorcontrib><creatorcontrib>AL-SABBAN, E</creatorcontrib><creatorcontrib>PRAGA, M</creatorcontrib><creatorcontrib>CASARI, G</creatorcontrib><creatorcontrib>BETTINELLI, A</creatorcontrib><creatorcontrib>COLUSSI, G</creatorcontrib><creatorcontrib>RODRIGUEZ-SORIANO, J</creatorcontrib><title>Paracellin-1, a renal tight junction protein required for paracellular Mg2+ resorption</title><title>Science (American Association for the Advancement of Science)</title><addtitle>Science</addtitle><description>Epithelia permit selective and regulated flux from apical to basolateral surfaces by transcellular passage through cells or paracellular flux between cells. 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These findings provide insight into Mg2+ homeostasis, demonstrate the role of a tight junction protein in human disease, and identify an essential component of a selective paracellular conductance.</description><subject>Amino Acid Sequence</subject><subject>Biological and medical sciences</subject><subject>Calcium - urine</subject><subject>Cell physiology</subject><subject>Cellular biology</subject><subject>Chromosomes, Human, Pair 3 - genetics</subject><subject>Claudins</subject><subject>Cloning</subject><subject>Cloning, Molecular</subject><subject>Coding</subject><subject>Female</subject><subject>Fundamental and applied biological sciences. 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Psychology</topic><topic>Genes</topic><topic>Genes, Recessive</topic><topic>Homeostasis</topic><topic>Humans</topic><topic>Kidney Diseases - genetics</topic><topic>Kidney Diseases - metabolism</topic><topic>Kidney Tubules - chemistry</topic><topic>Loop of Henle - chemistry</topic><topic>Loop of Henle - metabolism</topic><topic>Magnesium - blood</topic><topic>Magnesium - metabolism</topic><topic>Magnesium Deficiency - genetics</topic><topic>Magnesium Deficiency - metabolism</topic><topic>Male</topic><topic>Membrane and intracellular transports</topic><topic>Membrane Proteins - analysis</topic><topic>Membrane Proteins - chemistry</topic><topic>Membrane Proteins - genetics</topic><topic>Membrane Proteins - physiology</topic><topic>Molecular and cellular biology</topic><topic>Molecular Sequence Data</topic><topic>Mutation</topic><topic>Patients</topic><topic>Pedigree</topic><topic>Physical Chromosome Mapping</topic><topic>Proteins</topic><topic>Tight Junctions - metabolism</topic><topic>Vertebrates: urinary system</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>SIMON, D. 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| fulltext | fulltext |
| identifier | ISSN: 0036-8075 |
| ispartof | Science (American Association for the Advancement of Science), 1999-07, Vol.285 (5424), p.103-106 |
| issn | 0036-8075 1095-9203 |
| language | eng |
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| source | MEDLINE; JSTOR Archive Collection A-Z Listing; American Association for the Advancement of Science |
| subjects | Amino Acid Sequence Biological and medical sciences Calcium - urine Cell physiology Cellular biology Chromosomes, Human, Pair 3 - genetics Claudins Cloning Cloning, Molecular Coding Female Fundamental and applied biological sciences. Psychology Genes Genes, Recessive Homeostasis Humans Kidney Diseases - genetics Kidney Diseases - metabolism Kidney Tubules - chemistry Loop of Henle - chemistry Loop of Henle - metabolism Magnesium - blood Magnesium - metabolism Magnesium Deficiency - genetics Magnesium Deficiency - metabolism Male Membrane and intracellular transports Membrane Proteins - analysis Membrane Proteins - chemistry Membrane Proteins - genetics Membrane Proteins - physiology Molecular and cellular biology Molecular Sequence Data Mutation Patients Pedigree Physical Chromosome Mapping Proteins Tight Junctions - metabolism Vertebrates: urinary system |
| title | Paracellin-1, a renal tight junction protein required for paracellular Mg2+ resorption |
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