Pathogenesis of mitral valve disease in mucopolysaccharidosis VII dogs
PW Bigg, G Baldo, MM Sleeper, PA O'Donnell… - Molecular genetics and …, 2013 - Elsevier
Abstract Mucopolysaccharidosis VII (MPS VII) is due to the deficient activity of β-
glucuronidase (GUSB) and results in the accumulation of glycosaminoglycans (GAGs) in
lysosomes and multisystemic disease with cardiovascular manifestations. The goal here
was to determine the pathogenesis of mitral valve (MV) disease in MPS VII dogs. Untreated
MPS VII dogs had a marked reduction in the histochemical signal for structurally-intact
collagen in the MV at 6 months of age, when mitral regurgitation had developed. Electron …
glucuronidase (GUSB) and results in the accumulation of glycosaminoglycans (GAGs) in
lysosomes and multisystemic disease with cardiovascular manifestations. The goal here
was to determine the pathogenesis of mitral valve (MV) disease in MPS VII dogs. Untreated
MPS VII dogs had a marked reduction in the histochemical signal for structurally-intact
collagen in the MV at 6 months of age, when mitral regurgitation had developed. Electron …