The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy

Detalhes bibliográficos
Autor(a) principal: Wiesinger, Anna-Maria
Data de Publicação: 2022
Outros Autores: Bigger, Brian, Giugliani, Roberto, Scarpa, Maurizio, Moser, Tobias, Lampe, Christina, Kampmann, Christoph, Lagler, Florian B.
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Institucional da UFRGS
Texto Completo: http://hdl.handle.net/10183/245581
Resumo: Mucopolysaccharidoses (MPS) are a group of lysosomal storage diseases (LSDs), characterized by the accumulation of glycosaminoglycans (GAGs). GAG storageinduced inflammatory processes are a driver of cytopathology in MPS and pharmacological immunomodulation can bring improvements in brain, cartilage and bone pathology in rodent models. This manuscript reviews current knowledge with regard to inflammation in MPS patients and provides hypotheses for the therapeutic use of immunomodulators in MPS. Thus, we aim to set the foundation for a rational repurposing of the discussed molecules to minimize the clinical unmet needs still remaining despite enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT).
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spelling Wiesinger, Anna-MariaBigger, BrianGiugliani, RobertoScarpa, MaurizioMoser, TobiasLampe, ChristinaKampmann, ChristophLagler, Florian B.2022-07-28T04:44:46Z20221663-9812http://hdl.handle.net/10183/245581001145359Mucopolysaccharidoses (MPS) are a group of lysosomal storage diseases (LSDs), characterized by the accumulation of glycosaminoglycans (GAGs). GAG storageinduced inflammatory processes are a driver of cytopathology in MPS and pharmacological immunomodulation can bring improvements in brain, cartilage and bone pathology in rodent models. This manuscript reviews current knowledge with regard to inflammation in MPS patients and provides hypotheses for the therapeutic use of immunomodulators in MPS. Thus, we aim to set the foundation for a rational repurposing of the discussed molecules to minimize the clinical unmet needs still remaining despite enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT).application/pdfengFrontiers in pharmacology. Lausanne. Vol. 13 (May 2022), 863667, 20 p.MucopolissacaridosesInflamaçãoPatologiaTratamento farmacológicoMucopolysaccharidosesMPSImmunomodulationInflammationCytopathologyDrug discoveryThe inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapyEstrangeiroinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFRGSinstname:Universidade Federal do Rio Grande do Sul (UFRGS)instacron:UFRGSTEXT001145359.pdf.txt001145359.pdf.txtExtracted Texttext/plain117729http://www.lume.ufrgs.br/bitstream/10183/245581/2/001145359.pdf.txt6525d73ad0feb8d6c66dfa64144fe30dMD52ORIGINAL001145359.pdfTexto completo (inglês)application/pdf1387132http://www.lume.ufrgs.br/bitstream/10183/245581/1/001145359.pdf4882318a796cabb03518de521e1120a6MD5110183/2455812022-07-29 04:49:09.717825oai:www.lume.ufrgs.br:10183/245581Repositório de PublicaçõesPUBhttps://lume.ufrgs.br/oai/requestopendoar:2022-07-29T07:49:09Repositório Institucional da UFRGS - Universidade Federal do Rio Grande do Sul (UFRGS)false
dc.title.pt_BR.fl_str_mv The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
title The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
spellingShingle The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
Wiesinger, Anna-Maria
Mucopolissacaridoses
Inflamação
Patologia
Tratamento farmacológico
Mucopolysaccharidoses
MPS
Immunomodulation
Inflammation
Cytopathology
Drug discovery
title_short The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
title_full The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
title_fullStr The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
title_full_unstemmed The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
title_sort The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
author Wiesinger, Anna-Maria
author_facet Wiesinger, Anna-Maria
Bigger, Brian
Giugliani, Roberto
Scarpa, Maurizio
Moser, Tobias
Lampe, Christina
Kampmann, Christoph
Lagler, Florian B.
author_role author
author2 Bigger, Brian
Giugliani, Roberto
Scarpa, Maurizio
Moser, Tobias
Lampe, Christina
Kampmann, Christoph
Lagler, Florian B.
author2_role author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Wiesinger, Anna-Maria
Bigger, Brian
Giugliani, Roberto
Scarpa, Maurizio
Moser, Tobias
Lampe, Christina
Kampmann, Christoph
Lagler, Florian B.
dc.subject.por.fl_str_mv Mucopolissacaridoses
Inflamação
Patologia
Tratamento farmacológico
topic Mucopolissacaridoses
Inflamação
Patologia
Tratamento farmacológico
Mucopolysaccharidoses
MPS
Immunomodulation
Inflammation
Cytopathology
Drug discovery
dc.subject.eng.fl_str_mv Mucopolysaccharidoses
MPS
Immunomodulation
Inflammation
Cytopathology
Drug discovery
description Mucopolysaccharidoses (MPS) are a group of lysosomal storage diseases (LSDs), characterized by the accumulation of glycosaminoglycans (GAGs). GAG storageinduced inflammatory processes are a driver of cytopathology in MPS and pharmacological immunomodulation can bring improvements in brain, cartilage and bone pathology in rodent models. This manuscript reviews current knowledge with regard to inflammation in MPS patients and provides hypotheses for the therapeutic use of immunomodulators in MPS. Thus, we aim to set the foundation for a rational repurposing of the discussed molecules to minimize the clinical unmet needs still remaining despite enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT).
publishDate 2022
dc.date.accessioned.fl_str_mv 2022-07-28T04:44:46Z
dc.date.issued.fl_str_mv 2022
dc.type.driver.fl_str_mv Estrangeiro
info:eu-repo/semantics/article
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dc.identifier.uri.fl_str_mv http://hdl.handle.net/10183/245581
dc.identifier.issn.pt_BR.fl_str_mv 1663-9812
dc.identifier.nrb.pt_BR.fl_str_mv 001145359
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dc.language.iso.fl_str_mv eng
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dc.relation.ispartof.pt_BR.fl_str_mv Frontiers in pharmacology. Lausanne. Vol. 13 (May 2022), 863667, 20 p.
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reponame_str Repositório Institucional da UFRGS
collection Repositório Institucional da UFRGS
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