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- W2970599526 endingPage "165539" @default.
- W2970599526 startingPage "165539" @default.
- W2970599526 abstract "Heparan sulfate proteoglycans (HSPGs) are complex carbohydrate-modified proteins ubiquitously expressed on cell surfaces, extracellular matrix and basement membrane of mammalian tissues. Beside to serve as structural constituents, they regulate multiple cellular activities. A critical involvement of HSPGs in development has been established, and perturbations of HSPG-dependent pathways are associated with many human diseases. Recent evidence suggest a role of HSPGs in the pathogenesis of mucopolysaccharidoses (MPSs) where the accumulation of undigested HS results in the loss of cellular functions, tissue damage and organ dysfunctions accounting for clinical manifestations which include central nervous system (CNS) involvement, degenerative joint disease and reduced bone growth. Current therapies are not curative but only ameliorate the disease symptoms. Here, we highlight the link between HSPG functions in the development of CNS and musculoskeletal structures and the etiology of some MPS phenotypes, suggesting that HSPGs may represent potential targets for the therapy of such incurable diseases." @default.
- W2970599526 created "2019-09-05" @default.
- W2970599526 creator A5029582579 @default.
- W2970599526 creator A5038122177 @default.
- W2970599526 date "2019-11-01" @default.
- W2970599526 modified "2023-10-13" @default.
- W2970599526 title "Heparan sulfate proteoglycans: The sweet side of development turns sour in mucopolysaccharidoses" @default.
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