Article
Cardiac pathology in mucopolysaccharidosis I mice: Losartan modifies ERK1/2 activation during cardiac remodeling.
Journal of inherited metabolic disease - 1 May 2021
Gonzalez Esteban Alberto, Tobar Leitão Santiago Alonso, Soares Douglas Dos Santos, Tavares Angela Maria Vicente, Giugliani Roberto, Baldo Guilherme, Matte Ursula
Abstract excerpt
Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder caused by mutations in the IDUA gene, that codifies the alpha-L-iduronidase enzyme, which deficiency leads to storage of glycosaminoglycans, with multiple clinical manifestations. One of the leading causes of death in MPS I patients are cardiac complications such as cardiac valve thickening, conduction abnormalities, myocardial dysfunction, and...
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