The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis.
cysteine proteases
glycosaminoglycans
inhibition
mucopolysaccharidosis
proteolysis
Journal
Biomedicines
ISSN: 2227-9059
Titre abrégé: Biomedicines
Pays: Switzerland
ID NLM: 101691304
Informations de publication
Date de publication:
07 Mar 2023
07 Mar 2023
Historique:
received:
02
02
2023
revised:
27
02
2023
accepted:
04
03
2023
medline:
30
3
2023
entrez:
29
3
2023
pubmed:
30
3
2023
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 36979788
pii: biomedicines11030810
doi: 10.3390/biomedicines11030810
pmc: PMC10045161
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
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