Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion

Bibliographic Details
Main Author: Peixoto, Vanda
Publication Date: 2018
Other Authors: Vieira da Silva, Manuela, Prudêncio, Cristina
Language: eng
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: http://hdl.handle.net/10400.22/25679
Summary: Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare, acquired clonal disease of bone marrow stem-cells, genetically characterized by the somatic mutation of the phosphatidylinositol glycan class A (PIG-A) gene. That leads to defective synthesis of glycosylphosphatidylinositol (GPI) responsible for anchorage and fixation of surface proteins like complement decay-accelerating factor (DAF/CD55) and membrane inhibitor of reactive lysis (MIRL/CD59). These proteins protect red blood cells from lysis by activated complement, leading to intravascular hemolysis.
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spelling Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansionParoxysmal nocturnal hemoglobinuriaPIG-A geneClonal expansionParoxysmal Nocturnal Hemoglobinuria (PNH) is a rare, acquired clonal disease of bone marrow stem-cells, genetically characterized by the somatic mutation of the phosphatidylinositol glycan class A (PIG-A) gene. That leads to defective synthesis of glycosylphosphatidylinositol (GPI) responsible for anchorage and fixation of surface proteins like complement decay-accelerating factor (DAF/CD55) and membrane inhibitor of reactive lysis (MIRL/CD59). These proteins protect red blood cells from lysis by activated complement, leading to intravascular hemolysis.Escola Superior de Saúde P.PortoREPOSITÓRIO P.PORTOPeixoto, VandaVieira da Silva, ManuelaPrudêncio, Cristina2024-06-17T14:32:33Z2018-05-182018-05-18T00:00:00Zconference objectinfo:eu-repo/semantics/publishedVersionapplication/pdfhttp://hdl.handle.net/10400.22/25679eng978-989-20-8533-3info:eu-repo/semantics/openAccessreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAP2025-03-07T10:23:40Zoai:recipp.ipp.pt:10400.22/25679Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-29T00:52:05.997068Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion
title Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion
spellingShingle Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion
Peixoto, Vanda
Paroxysmal nocturnal hemoglobinuria
PIG-A gene
Clonal expansion
title_short Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion
title_full Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion
title_fullStr Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion
title_full_unstemmed Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion
title_sort Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion
author Peixoto, Vanda
author_facet Peixoto, Vanda
Vieira da Silva, Manuela
Prudêncio, Cristina
author_role author
author2 Vieira da Silva, Manuela
Prudêncio, Cristina
author2_role author
author
dc.contributor.none.fl_str_mv REPOSITÓRIO P.PORTO
dc.contributor.author.fl_str_mv Peixoto, Vanda
Vieira da Silva, Manuela
Prudêncio, Cristina
dc.subject.por.fl_str_mv Paroxysmal nocturnal hemoglobinuria
PIG-A gene
Clonal expansion
topic Paroxysmal nocturnal hemoglobinuria
PIG-A gene
Clonal expansion
description Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare, acquired clonal disease of bone marrow stem-cells, genetically characterized by the somatic mutation of the phosphatidylinositol glycan class A (PIG-A) gene. That leads to defective synthesis of glycosylphosphatidylinositol (GPI) responsible for anchorage and fixation of surface proteins like complement decay-accelerating factor (DAF/CD55) and membrane inhibitor of reactive lysis (MIRL/CD59). These proteins protect red blood cells from lysis by activated complement, leading to intravascular hemolysis.
publishDate 2018
dc.date.none.fl_str_mv 2018-05-18
2018-05-18T00:00:00Z
2024-06-17T14:32:33Z
dc.type.driver.fl_str_mv conference object
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10400.22/25679
url http://hdl.handle.net/10400.22/25679
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 978-989-20-8533-3
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Escola Superior de Saúde P.Porto
publisher.none.fl_str_mv Escola Superior de Saúde P.Porto
dc.source.none.fl_str_mv reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron:RCAAP
instname_str FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron_str RCAAP
institution RCAAP
reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
collection Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
repository.name.fl_str_mv Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
repository.mail.fl_str_mv info@rcaap.pt
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