Cardiac pathology in mucopolysaccharidosis I mice: Losartan modifies ERK1/2 activation during cardiac remodeling

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 patient...

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Bibliographic Details
Published in:	Journal of inherited metabolic disease Vol. 44; no. 3; pp. 740 - 750
Main Authors:	Gonzalez, Esteban Alberto, Tobar Leitão, Santiago Alonso, Soares, Douglas dos Santos, Tavares, Angela Maria Vicente, Giugliani, Roberto, Baldo, Guilherme, Matte, Ursula
Format:	Journal Article
Language:	English
Published:	Hoboken, USA John Wiley & Sons, Inc 01-05-2021 Blackwell Publishing Ltd
Subjects:	AKT AKT protein Animals cardiac disease cardiac remodeling Cathepsins Coronary artery disease Disease Models, Animal Echocardiography ERK1/2 Extracellular signal-regulated kinase Female Genetic disorders Glycosaminoglycans Heart diseases Heart Diseases - drug therapy Heart Diseases - genetics Heart Diseases - pathology Hypertrophy IDUA gene Iduronidase - genetics losartan Losartan - pharmacology Male MAP Kinase Signaling System - drug effects MAP Kinase Signaling System - genetics Metabolic disorders Metalloproteinase Mice Mice, Inbred C57BL Mucopolysaccharidosis Mucopolysaccharidosis I - drug therapy Mucopolysaccharidosis I - genetics Mucopolysaccharidosis I - pathology mucopolysaccharidosis type I Mutation Propranolol Recovery of function Rheumatic heart disease Ventricular Remodeling - drug effects ERK1/2 cardiac remodeling propranolol cardiac disease AKT losartan mucopolysaccharidosis type I
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