Genome editing in mucopolysaccharidoses and mucolipidoses.


Journal

Progress in molecular biology and translational science
ISSN: 1878-0814
Titre abrégé: Prog Mol Biol Transl Sci
Pays: Netherlands
ID NLM: 101498165

Informations de publication

Date de publication:
2021
Historique:
entrez: 27 6 2021
pubmed: 28 6 2021
medline: 26 11 2021
Statut: ppublish

Résumé

Mucopolysaccharidoses (MPS) and mucolipidoses (ML) are disorders that alter lysosome function. While MPS are caused by mutation in enzymes that degrade glycosaminoglycans, the ML are disorders characterized by reduced function in the phosphotransferase enzyme. Multiple clinical features are associated with these diseases and the exact mechanisms that could explain such different clinical manifestations in patients are still unknown. Furthermore, there are no curative treatment for any of MPS and ML conditions so far. Gene editing holds promise as a tool for the creation of cell and animal models to help explain disease pathogenesis, as well as a platform for gene therapy. In this chapter, we discuss the main studies involving genome editing for MPS and the prospect applications for ML.

Identifiants

pubmed: 34175047
pii: S1877-1173(21)00037-5
doi: 10.1016/bs.pmbts.2021.01.026
pii:
doi:

Substances chimiques

Glycosaminoglycans 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

327-351

Informations de copyright

Copyright © 2021 Elsevier Inc. All rights reserved.

Auteurs

Hallana Souza Santos (HS)

Laboratório Células, Tecidos e Genes do Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.

Edina Poletto (E)

Laboratório Células, Tecidos e Genes do Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.

Roselena Schuh (R)

Laboratório Células, Tecidos e Genes do Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.

Ursula Matte (U)

Laboratório Células, Tecidos e Genes do Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.

Guilherme Baldo (G)

Laboratório Células, Tecidos e Genes do Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address: gbaldo@hcpa.edu.br.

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Classifications MeSH