Mucopolysaccharidosis VII

Known as: VIIs, Mucopolysaccharidosis, MPS VII, Deficiencies, beta-Glucuronidase 
A rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme beta-glucuronidase. It is characterized by hepatosplenomegaly… (More)
National Institutes of Health

Papers overview

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2009
2009
Mucopolysaccharidosis VII (MPS VII) is a lysosomal storage disease in which deficiency in beta-glucuronidase results in… (More)
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Highly Cited
2006
Highly Cited
2006
Purpose: To evaluate the safety and efficacy of recombinant human iduronate-2-sulfatase (idursulfase) in the treatment of… (More)
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Highly Cited
2005
Highly Cited
2005
Enzyme replacement therapy (ERT) effectively reverses storage in several lysosomal storage diseases. However, improvement in… (More)
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Highly Cited
2002
Highly Cited
2002
Dogs with mucopolysaccharidosis VII (MPS VII) were injected intravenously at 2-3 days of age with a retroviral vector (RV… (More)
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2002
2002
Mucopolysaccharidosis VII (MPS VII) is a lysosomal storage disease caused by beta-glucuronidase (GUSB) deficiency. Intravenous… (More)
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Highly Cited
1995
Highly Cited
1995
MANY metabolic diseases affecting the central nervous system are refractory to treatment because the blood–brain barrier… (More)
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Highly Cited
1994
Highly Cited
1994
Recombinant mouse beta-glucuronidase administered intravenously to newborn mice with mucopolysaccharidosis type VII (MPS VII) is… (More)
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Highly Cited
1993
Highly Cited
1993
BACKGROUND Bone marrow transplantation (BMT) proved an effective therapy for murine mucopolysaccharidosis type VII (MPS VII) in… (More)
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Highly Cited
1989
Highly Cited
1989
We have characterized a new mutant mouse that has virtually no beta-glucuronidase activity. This biochemical defect causes a… (More)
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1984
1984
Summary: This report describes a third mucopolysaccharidosis in animals: canine mucopolysaccharidosis VII. The affected dog was… (More)
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