Oxidative profile exhibited by Mucopolysaccharidosis type IVA patients at diagnosis: Increased keratan urinary levels

Oxidative profile exhibited by Mucopolysaccharidosis type IVA patients at diagnosis: Increased keratan urinary levels

Morquio A disease (Mucopolysaccharidosis type IVA, MPS IVA) is one of the 11 mucopolysaccharidoses (MPSs), a heterogeneous group of inherited lysosomal storage disorders (LSDs) caused by deficiency in enzymes need to degrade glycosaminoglycans (GAGs). Morquio A is characterized by a decrease in N-ac...

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Bibliographic Details
Main Authors: Bruna Donida, Desirèe P. Marchetti, Carlos Eduardo Diaz Jacques, Graziela Ribas, Marion Deon, Paula Manini, Helen Tais da Rosa, Dinara Jaqueline Moura, Jenifer Saffi, Roberto Giugliani, Carmen Regla Vargas
Format: Article
Language:English
Published: Elsevier 2017-06-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Mucopolysaccharidosis type IVA
Morquio A syndrome
Oxidative stress
Keratan sulfate
N-acetyl-galactosamine-6-sulfatase
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426917300265

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http://www.sciencedirect.com/science/article/pii/S2214426917300265

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