Cargando…

The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis

Mucopolysaccharidosis (MPS) consists of a group of inherited lysosomal storage disorders that are caused by a defect of certain enzymes that participate in the metabolism of glycosaminoglycans (GAGs). The abnormal accumulation of GAGs leads to progressive dysfunctions in various tissues and organs d...

Descripción completa

Detalles Bibliográficos
Autores principales: David, Alexis, Chazeirat, Thibault, Saidi, Ahlame, Lalmanach, Gilles, Lecaille, Fabien
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10045161/
https://www.ncbi.nlm.nih.gov/pubmed/36979788
http://dx.doi.org/10.3390/biomedicines11030810
_version_ 1784913533143613440
author David, Alexis
Chazeirat, Thibault
Saidi, Ahlame
Lalmanach, Gilles
Lecaille, Fabien
author_facet David, Alexis
Chazeirat, Thibault
Saidi, Ahlame
Lalmanach, Gilles
Lecaille, Fabien
author_sort David, Alexis
collection PubMed
description Mucopolysaccharidosis (MPS) consists of a group of inherited lysosomal storage disorders that are caused by a defect of certain enzymes that participate in the metabolism of glycosaminoglycans (GAGs). The abnormal accumulation of GAGs leads to progressive dysfunctions in various tissues and organs during childhood, contributing to premature death. As the current therapies are limited and inefficient, exploring the molecular mechanisms of the pathology is thus required to address the unmet needs of MPS patients to improve their quality of life. Lysosomal cysteine cathepsins are a family of proteases that play key roles in numerous physiological processes. Dysregulation of cysteine cathepsins expression and activity can be frequently observed in many human diseases, including MPS. This review summarizes the basic knowledge on MPS disorders and their current management and focuses on GAGs and cysteine cathepsins expression in MPS, as well their interplay, which may lead to the development of MPS-associated disorders.
format Online
Article
Text
id pubmed-10045161
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-100451612023-03-29 The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis David, Alexis Chazeirat, Thibault Saidi, Ahlame Lalmanach, Gilles Lecaille, Fabien Biomedicines Review Mucopolysaccharidosis (MPS) consists of a group of inherited lysosomal storage disorders that are caused by a defect of certain enzymes that participate in the metabolism of glycosaminoglycans (GAGs). The abnormal accumulation of GAGs leads to progressive dysfunctions in various tissues and organs during childhood, contributing to premature death. As the current therapies are limited and inefficient, exploring the molecular mechanisms of the pathology is thus required to address the unmet needs of MPS patients to improve their quality of life. Lysosomal cysteine cathepsins are a family of proteases that play key roles in numerous physiological processes. Dysregulation of cysteine cathepsins expression and activity can be frequently observed in many human diseases, including MPS. This review summarizes the basic knowledge on MPS disorders and their current management and focuses on GAGs and cysteine cathepsins expression in MPS, as well their interplay, which may lead to the development of MPS-associated disorders. MDPI 2023-03-07 /pmc/articles/PMC10045161/ /pubmed/36979788 http://dx.doi.org/10.3390/biomedicines11030810 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
David, Alexis
Chazeirat, Thibault
Saidi, Ahlame
Lalmanach, Gilles
Lecaille, Fabien
The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis
title The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis
title_full The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis
title_fullStr The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis
title_full_unstemmed The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis
title_short The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis
title_sort interplay of glycosaminoglycans and cysteine cathepsins in mucopolysaccharidosis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10045161/
https://www.ncbi.nlm.nih.gov/pubmed/36979788
http://dx.doi.org/10.3390/biomedicines11030810
work_keys_str_mv AT davidalexis theinterplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT chazeiratthibault theinterplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT saidiahlame theinterplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT lalmanachgilles theinterplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT lecaillefabien theinterplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT davidalexis interplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT chazeiratthibault interplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT saidiahlame interplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT lalmanachgilles interplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis
AT lecaillefabien interplayofglycosaminoglycansandcysteinecathepsinsinmucopolysaccharidosis