Neuropathology in Mouse Models of Mucopolysaccharidosis Type I, IIIA and IIIB

Hyperactive behaviour in the mouse model of mucopolysaccharidosis IIIB in the open field and home cage environments

(2011) A. Langford-Smith et al.   GENES BRAIN AND BEHAVIOR

Mucopolysaccharidosis Type I, Unique Structure of Accumulated Heparan Sulfate and IncreasedN-Sulfotransferase Activity in Mice Lacking α-l-iduronidase

(2011) Rebecca J. Holley et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Female Mucopolysaccharidosis IIIA Mice Exhibit Hyperactivity and a Reduced Sense of Danger in the Open Field Test

(2011) Alex Langford-Smith et al.   PLoS One

Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB

(2010) Maria M. Canal et al.   BEHAVIOURAL BRAIN RESEARCH

Genistein Improves Neuropathology and Corrects Behaviour in a Mouse Model of Neurodegenerative Metabolic Disease

(2010) Marcelina Malinowska et al.   PLoS One

Examination of intravenous and intra-CSF protein delivery for treatment of neurological disease

(2009) Kim M. Hemsley et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Inflammation Triggers Synaptic Alteration and Degeneration in Experimental Autoimmune Encephalomyelitis

(2009) D. Centonze et al.   JOURNAL OF NEUROSCIENCE

Enhanced degradation of synaptophysin by the proteasome in mucopolysaccharidosis type IIIB

(2009) Sandrine Vitry et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Genistein reduces lysosomal storage in peripheral tissues of mucopolysaccharide IIIB mice

(2009) Marcelina Malinowska et al.   MOLECULAR GENETICS AND METABOLISM

Validation of disaccharide compositions derived from dermatan sulfate and heparan sulfate in mucopolysaccharidoses and mucolipidoses II and III by tandem mass spectrometry

(2009) Shunji Tomatsu et al.   MOLECULAR GENETICS AND METABOLISM

Secondary lipid accumulation in lysosomal disease

(2008) Steven U. Walkley et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Unrelated donor umbilical cord blood transplantation for inherited metabolic disorders in 159 pediatric patients from a single center: influence of cellular composition of the graft on transplantation outcomes

(2008) V. K. Prasad et al.   BLOOD

The mucopolysaccharidoses: a success of molecular medicine

(2008) Lorne A. Clarke   EXPERT REVIEWS IN MOLECULAR MEDICINE

A simplified and sensitive fluorescent method for disaccharide analysis of both heparan sulfate and chondroitin/dermatan sulfates from biological samples

(2008) J. A Deakin et al.   GLYCOBIOLOGY

Sanfilippo syndrome: A mini-review

(2008) M. J. Valstar et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Developmental roles for Homer: more than just a pretty scaffold

(2008) Lisa Foa et al.   JOURNAL OF NEUROCHEMISTRY

Innate and adaptive immune activation in the brain of MPS IIIB mouse model

(2008) Julianne DiRosario et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Early Neurodegeneration Progresses Independently of Microglial Activation by Heparan Sulfate in the Brain of Mucopolysaccharidosis IIIB Mice

(2008) Jérôme Ausseil et al.   PLoS One