Biomechanical and histological characterization of MPS I mice femurs.
Sign Up to like & getrecommendations! Published in 2021 at "Acta histochemica"
DOI: 10.1016/j.acthis.2020.151678
Abstract: Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder characterized by alpha-L-iduronidase (IDUA) deficiency, an enzyme responsible for glycosaminoglycan degradation. Musculoskeletal impairment is an important component of the morbidity related to the disease, as… read more here.
Keywords: month old; biomechanical histological; idua; mice ... See more keywords
RTB lectin-mediated delivery of lysosomal α-l-iduronidase mitigates disease manifestations systemically including the central nervous system.
Sign Up to like & getrecommendations! Published in 2018 at "Molecular genetics and metabolism"
DOI: 10.1016/j.ymgme.2017.11.013
Abstract: Mucopolysaccharidosis type I (MPS I) is a lysosomal disease resulting from deficiency in the α-L-iduronidase (IDUA) hydrolase and subsequent accumulation of glycosaminoglycan (GAG). Clinically, enzyme replacement therapy (ERT) with IDUA achieves negligible neurological benefits presumably… read more here.
Keywords: mediated delivery; idua; delivery; lectin ... See more keywords