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Induced Pluripotent Stem Cells to Understand Mucopolysaccharidosis. I: Demonstration of a Migration Defect in Neural Precursors.

Cells - 3 Dec 2020

Lito Silvin, Sidibe Adama, Ilmjarv Sten, Burda Patricie, Baumgartner Matthias, Wehrle-Haller Bernhard, Krause Karl-Heinz, Marteyn Antoine

Abstract excerpt

Background: Mucopolysaccharidosis type I-Hurler (MPS1-H) is a severe genetic lysosomal storage disorder due to loss-of-function mutations in the IDUA gene. The subsequent complete deficiency of alpha l-iduronidase enzyme is directly responsible of a progressive accumulation of glycosaminoglycans (GAG) in lysosomes which affects the functions of many tissues. Consequently, MPS1 is characterized by systemic...

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Cell Differentiation
Cell Movement
Glycosaminoglycans
Iduronidase
Induced Pluripotent Stem Cells

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Mucopolysaccharidosis I

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PMID: 33287330
DOI: 10.3390/cells9122593
PMCID: PMC7761689

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Induced Pluripotent Stem Cells to Understand Mucopolysaccharidosis. I: Demonstration of a Migration Defect in Neural Precursors.PMID 33287330Read ↗

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