The Interplay of Glycosaminoglycans and Cysteine Cathepsins in Mucopolysaccharidosis.

ABSTRACT: 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 revie

SUBMITTER: David A 

PROVIDER: S-EPMC10045161 | biostudies-literature | 2023 Mar

REPOSITORIES: biostudies-literature