Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis

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 pos...

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Autores principales:	Vittori, Daniela Cecilia, Nesse, Alcira Beatriz
Publicado:	2016
Materias:	Aquaporin-1 Erythropoietin Serum osmolality Spherocytosis ankyrin aquaporin 1 erythrocyte band 4.1 protein erythrocyte band 4.2 protein erythropoietin spectrin 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 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 Gene Expression Regulation Hemolysis Heterozygote Humans Leukemia, Lymphocytic, Chronic, B-Cell Middle Aged Osmolar Concentration Osmotic Fragility Splenectomy
Acceso en línea:	https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_09395555_v95_n10_p1595_Crisp http://hdl.handle.net/20.500.12110/paper_09395555_v95_n10_p1595_Crisp
Aporte de:	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires

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spelling	paper:paper_09395555_v95_n10_p1595_Crisp2023-06-08T15:53:29Z Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis Vittori, Daniela Cecilia Nesse, Alcira Beatriz 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 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. Fil:Vittori, D.C. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Nesse, A. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. 2016 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_09395555_v95_n10_p1595_Crisp http://hdl.handle.net/20.500.12110/paper_09395555_v95_n10_p1595_Crisp
institution	Universidad de Buenos Aires
institution_str	I-28
repository_str	R-134
collection	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA)
topic	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
spellingShingle	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 Vittori, Daniela Cecilia Nesse, Alcira Beatriz Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis
topic_facet	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
description	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.
author	Vittori, Daniela Cecilia Nesse, Alcira Beatriz
author_facet	Vittori, Daniela Cecilia Nesse, Alcira Beatriz
author_sort	Vittori, Daniela Cecilia
title	Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis
title_short	Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis
title_full	Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis
title_fullStr	Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis
title_full_unstemmed	Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis
title_sort	red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis
publishDate	2016
url	https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_09395555_v95_n10_p1595_Crisp http://hdl.handle.net/20.500.12110/paper_09395555_v95_n10_p1595_Crisp
work_keys_str_mv	AT vittoridanielacecilia redbloodcellaquaporin1expressionisdecreasedinhereditaryspherocytosis AT nessealcirabeatriz redbloodcellaquaporin1expressionisdecreasedinhereditaryspherocytosis
_version_	1768543855451308032

Red blood cell aquaporin-1 expression is decreased in hereditary spherocytosis

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