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Crisp, R.L.; Maltaneri, R.E.; Vittori, D.C.; Solari, L.; Gammella, D.; Schvartzman, G.; García, E.; Rapetti, M.C.; Donato, H.; Nesse, A. "Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis" (2016) Annals of Hematology. 95(10):1595-1601
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Abstract:

Aquaporin-1 (AQP1) is the membrane water channel responsible for changes in erythrocyte volume in response to the tonicity of the medium. As the aberrant distribution of proteins in hereditary spherocytosis (HS) generates deficiencies of proteins other than those codified by the mutated gene, we postulated that AQP1 expression might be impaired in spherocytes. AQP1 expression was evaluated through flow cytometry in 5 normal controls, 1 autoimmune hemolytic anemia, 10 HS (2 mild, 3 moderate, 2 severe, and 3 splenectomized), and 3 silent carriers. The effect of AQP1 inhibitors was evaluated through water flow-based tests: osmotic fragility and hypertonic cryohemolysis. Serum osmolality was measured in 20 normal controls and 13 HS. The effect of erythropoietin (Epo) on AQP1 expression was determined in cultures of erythroleukemia UT-7 cells, dependent on Epo to survive. Independent of erythrocyte size, HS patients showed a lower content of AQP1 in erythrocyte membranes which correlated with the severity of the disease. Accordingly, red blood cells from HS subjects were less sensitive to cryohemolysis than normal erythrocytes after inhibition of the AQP1 water channel. A lower serum osmolality in HS with respect to normal controls suggests alterations during reticulocyte remodeling. The decreased AQP1 expression could contribute to explain variable degrees of anemia in hereditary spherocytosis. The finding of AQP1 expression induced by Epo in a model of erythroid cells may be interpreted as a mechanism to restore the balance of red cell water fluxes. © 2016, Springer-Verlag Berlin Heidelberg.

Registro:

Documento: Artículo
Título:Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis
Autor:Crisp, R.L.; Maltaneri, R.E.; Vittori, D.C.; Solari, L.; Gammella, D.; Schvartzman, G.; García, E.; Rapetti, M.C.; Donato, H.; Nesse, A.
Filiación:División Hematología Clínica, Departamento de Medicina, Hospital Nacional Alejandro Posadas, Buenos Aires, Argentina
Departamento de Química Biológica, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Buenos Aires, Argentina
Consultorios de Hematología Infantil, Buenos Aires, Argentina
IQUIBICEN-CONICET (Consejo Nacional de Investigaciones Científicas y Técnicas), Pabellón II, Piso 4, Ciudad Universitaria, Ciudad Autónoma de Buenos Aires, C1428EHA, Argentina
Laboratorio de Citometría, Departamento de Diagnóstico, Hospital Nacional Alejandro Posadas, Buenos Aires, Argentina
Servicio de Oncohematología Pediátrica, Departamento de Pediatría, Hospital Nacional Alejandro Posadas, Buenos Aires, Argentina
Sección Hematología/Oncología, Hospital Municipal del Niño de San Justo, San Justo, Buenos Aires, Argentina
Palabras clave:Aquaporin-1; Erythropoietin; Serum osmolality; Spherocytosis; ankyrin; aquaporin 1; erythrocyte band 4.1 protein; erythrocyte band 4.2 protein; erythropoietin; spectrin; aquaporin 1; erythropoietin; Article; autoimmune hemolytic anemia; clinical article; controlled study; erythrocyte; erythrocyte membrane; erythrocyte volume; erythroid precursor cell; erythroleukemia cell; flow cytometry; hereditary spherocytosis; human; human cell; polyacrylamide gel electrophoresis; priority journal; protein expression; reticulocyte; serum osmolality; adolescent; adult; biosynthesis; blood; body water; cell line; child; chronic lymphatic leukemia; erythrocyte; gene expression regulation; genetics; hemolysis; heterozygote; metabolism; middle aged; osmolarity; osmotic fragility; pathology; preschool child; Spherocytosis, Hereditary; splenectomy; transport at the cellular level; Adolescent; Adult; Anemia, Hemolytic, Autoimmune; Aquaporin 1; Biological Transport; Body Water; Cell Line; Child; Child, Preschool; Erythrocyte Membrane; Erythrocytes; Erythropoietin; Gene Expression Regulation; Hemolysis; Heterozygote; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Middle Aged; Osmolar Concentration; Osmotic Fragility; Spherocytosis, Hereditary; Splenectomy
Año:2016
Volumen:95
Número:10
Página de inicio:1595
Página de fin:1601
DOI: http://dx.doi.org/10.1007/s00277-016-2757-0
Título revista:Annals of Hematology
Título revista abreviado:Ann. Hematol.
ISSN:09395555
CODEN:ANHEE
CAS:aquaporin 1, 146410-94-8, 149348-86-7; erythropoietin, 11096-26-7; spectrin, 12634-43-4; Aquaporin 1; Erythropoietin
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_09395555_v95_n10_p1595_Crisp

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Citas:

---------- APA ----------
Crisp, R.L., Maltaneri, R.E., Vittori, D.C., Solari, L., Gammella, D., Schvartzman, G., García, E.,..., Nesse, A. (2016) . Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis. Annals of Hematology, 95(10), 1595-1601.
http://dx.doi.org/10.1007/s00277-016-2757-0
---------- CHICAGO ----------
Crisp, R.L., Maltaneri, R.E., Vittori, D.C., Solari, L., Gammella, D., Schvartzman, G., et al. "Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis" . Annals of Hematology 95, no. 10 (2016) : 1595-1601.
http://dx.doi.org/10.1007/s00277-016-2757-0
---------- MLA ----------
Crisp, R.L., Maltaneri, R.E., Vittori, D.C., Solari, L., Gammella, D., Schvartzman, G., et al. "Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis" . Annals of Hematology, vol. 95, no. 10, 2016, pp. 1595-1601.
http://dx.doi.org/10.1007/s00277-016-2757-0
---------- VANCOUVER ----------
Crisp, R.L., Maltaneri, R.E., Vittori, D.C., Solari, L., Gammella, D., Schvartzman, G., et al. Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis. Ann. Hematol. 2016;95(10):1595-1601.
http://dx.doi.org/10.1007/s00277-016-2757-0