The inflammation in the cytopathology of patients with mucopolysaccharidoses : immunomodulatory drugs as an approach to therapy
Autor(a) principal: | |
---|---|
Data de Publicação: | 2022 |
Outros Autores: | , , , , , , |
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). |
id |
UFRGS-2_d7b9cadf61b05eaeb3085793821d4380 |
---|---|
oai_identifier_str |
oai:www.lume.ufrgs.br:10183/245581 |
network_acronym_str |
UFRGS-2 |
network_name_str |
Repositório Institucional da UFRGS |
repository_id_str |
|
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 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
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 |
identifier_str_mv |
1663-9812 001145359 |
url |
http://hdl.handle.net/10183/245581 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.ispartof.pt_BR.fl_str_mv |
Frontiers in pharmacology. Lausanne. Vol. 13 (May 2022), 863667, 20 p. |
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.source.none.fl_str_mv |
reponame:Repositório Institucional da UFRGS instname:Universidade Federal do Rio Grande do Sul (UFRGS) instacron:UFRGS |
instname_str |
Universidade Federal do Rio Grande do Sul (UFRGS) |
instacron_str |
UFRGS |
institution |
UFRGS |
reponame_str |
Repositório Institucional da UFRGS |
collection |
Repositório Institucional da UFRGS |
bitstream.url.fl_str_mv |
http://www.lume.ufrgs.br/bitstream/10183/245581/2/001145359.pdf.txt http://www.lume.ufrgs.br/bitstream/10183/245581/1/001145359.pdf |
bitstream.checksum.fl_str_mv |
6525d73ad0feb8d6c66dfa64144fe30d 4882318a796cabb03518de521e1120a6 |
bitstream.checksumAlgorithm.fl_str_mv |
MD5 MD5 |
repository.name.fl_str_mv |
Repositório Institucional da UFRGS - Universidade Federal do Rio Grande do Sul (UFRGS) |
repository.mail.fl_str_mv |
|
_version_ |
1801225062620069888 |