Abstract
Mucopolysaccharidosis type I (MPSI) is an inborn error of metabolism of autosomal inheritance, with progressive character, due to the deficiency of the alpha-L-iduronidase enzyme, responsible for the degradation of glycosaminoglycans (GAGs) dermatam sulfate and heparam sulfate, leading to these substances storage in lysosomal. Lysosomal storage leads to various cellular and tissue dysfunc…