Sly syndrome

Sly syndrome
Other names	β-glucuronidase deficiency, β-glucuronidase deficiency mucopolysaccharidosis,
Sly syndrome has an autosomal recessive pattern of inheritance.
Specialty	Endocrinology

Sly syndrome, also called mucopolysaccharidosis type VII (MPS-VII), is an autosomal recessive lysosomal storage disease caused by a deficiency of the enzyme β-glucuronidase. This enzyme is responsible for breaking down large sugar molecules called glycosaminoglycans (AKA GAGs, or mucopolysaccharides). The inability to break down GAGs leads to a buildup in many tissues and organs of the body. The severity of the disease can vary widely.^[1]