http://purl.uniprot.org/citations/16174750 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/16174750 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/16174750 | http://www.w3.org/2000/01/rdf-schema#comment | "The multisubunit vacuolar-type H(+)ATPases mediate acidification of various intracellular organelles and in some tissues mediate H(+) secretion across the plasma membrane. Mutations in the B1-subunit of the apical H(+)ATPase that secretes protons in the distal nephron cause distal renal tubular acidosis in humans, a condition characterized by metabolic acidosis with an inappropriately alkaline urine. To examine the detailed cellular and organismal physiology resulting from this mutation, we have generated mice deficient in the B1-subunit (Atp6v1b1(-/-) mice). Urine pH is more alkaline and metabolic acidosis is more severe in Atp6v1b1(-/-) mice after oral acid challenge, demonstrating a failure of normal urinary acidification. In Atp6v1b1(-/-) mice, the normal urinary acidification induced by a lumen-negative potential in response to furosemide infusion is abolished. After an acute intracellular acidification, Na(+)-independent pH recovery rates of individual Atp6v1b1(-/-) intercalated cells of the cortical collecting duct are markedly reduced and show no further decrease after treatment with the selective H(+)ATPase inhibitor concanamycin. Apical expression of the alternative B-subunit isoform, B2, is increased in Atp6v1b1(-/-) medulla and colocalizes with the H(+)ATPase E-subunit; however, the greater severity of metabolic acidosis in Atp6v1b1(-/-) mice after oral acid challenge indicates that the B2-subunit cannot fully functionally compensate for the loss of B1. Our results indicate that the B1 isoform is the major B-subunit isoform that incorporates into functional, plasma membrane H(+)ATPases in intercalated cells of the cortical collecting duct and is required for maximal urinary acidification."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.org/dc/terms/identifier | "doi:10.1073/pnas.0506769102"xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.org/dc/terms/identifier | "doi:10.1073/pnas.0506769102"xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Brown D."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Brown D."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Wagner C.A."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Wagner C.A."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Lifton R.P."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Lifton R.P."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Breton S."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Breton S."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Bailey M.A."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Bailey M.A."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Giebisch G."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Giebisch G."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Finberg K.E."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Finberg K.E."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Geibel J.P."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Geibel J.P."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Paunescu T.G."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/author | "Paunescu T.G."xsd:string |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/date | "2005"xsd:gYear |
http://purl.uniprot.org/citations/16174750 | http://purl.uniprot.org/core/date | "2005"xsd:gYear |