Evidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice.

Behavioural Brain Research
Guilherme BaldoUrsula Matte

Abstract

Mucopolysaccharidosis (MPS) type I (Hurler syndrome) is a lysosomal storage disorder characterized by deficiency of alpha-L-iduronidase (IDUA), intracellular storage of glycosaminoglycans (GAGs) and progressive neurological pathology. The MPS I mouse model provides an opportunity to study the pathophysiology of this disorder and to determine the efficacy of novel therapies. Previous work has demonstrated a series of abnormalities in MPS I mice behavior, but so far some important brain functions have not been addressed. Therefore, in the present study we aimed to determine if MPS I mice have motor abnormalities, and at what age they become detectable. MPS I and normal male mice from 2 to 8 months of age were tested in open-field for locomotor activity, hindlimb gait analysis and hang wire performance. We were able to detect a progressive reduction in the crossings and rearings in the open field test and in the hang wire test in MPS I mice from 4 months, as well as a reduction in the gait length at 8 months. Histological examination of 8-month old mice cortex and cerebellum revealed storage of GAGs in Purkinje cells and neuroinflammation, evidenced by GFAP immunostaining. However TUNEL staining was negative, suggesting that death...Continue Reading

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Citations

Dec 30, 2014·Molecular Genetics and Metabolism·Guilherme BaldoRoberto Giugliani
Jun 22, 2013·International Journal of Experimental Pathology·Patricia G de OliveiraRicardo M Xavier
Apr 19, 2015·Journal of Controlled Release : Official Journal of the Controlled Release Society·Michelle FragaHelder Ferreira Teixeira
Dec 12, 2012·Behavioural Brain Research·Stijn StroobantsRudi D'Hooge
Jul 28, 2016·Translational Research : the Journal of Laboratory and Clinical Medicine·Ana Paula SchneiderGuilherme Baldo
Dec 14, 2019·PloS One·Ana Barbosa MendesVânia D'Almeida
Feb 7, 2020·Journal of Clinical Medicine·Gustavo M VianaAlexey V Pshezhetsky
Mar 1, 2020·Biomolecules·Valeria De PasqualeMarianna Caterino
Feb 26, 2020·International Journal of Molecular Sciences·Gustavo Monteiro VianaHelena Bonciani Nader
Jul 6, 2020·Metabolic Brain Disease·A S AzambujaGuilherme Baldo
Mar 13, 2020·Journal of Molecular Histology·Cinthia Castro do NascimentoVânia D'Almeida
Dec 16, 2020·Molecular Biology Reports·Cinthia Castro do NascimentoVânia D'Almeida
Aug 3, 2020·Molecular Genetics and Metabolism·Ainslie Derrick-RobertsSharon Byers

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