Mechanism of shortened bones in mucopolysaccharidosis VII
Mucopolysaccharidosis VII (MPS VII) is a lysosomal storage disease in which deficiency in β-
glucuronidase results in glycosaminoglycan (GAG) accumulation in and around cells,
causing shortened long bones through mechanisms that remain largely unclear. We
demonstrate here that MPS VII mice accumulate massive amounts of the GAG chondroitin-4-
sulfate (C4S) in their growth plates, the cartilaginous region near the ends of long bones
responsible for growth. MPS VII mice also have only 60% of the normal number of …
glucuronidase results in glycosaminoglycan (GAG) accumulation in and around cells,
causing shortened long bones through mechanisms that remain largely unclear. We
demonstrate here that MPS VII mice accumulate massive amounts of the GAG chondroitin-4-
sulfate (C4S) in their growth plates, the cartilaginous region near the ends of long bones
responsible for growth. MPS VII mice also have only 60% of the normal number of …
