mucopolysaccharidosis VI

Also found in: Dictionary, Thesaurus, Encyclopedia, Wikipedia.
Related to mucopolysaccharidosis VI: Maroteaux-Lamy syndrome

mucopolysaccharidosis VI

Maroteaux-Lamy syndrome; MPS due to a deficiency of the enzyme N-acetylgalactosamine-4-sulfatase. Clinically, MPS VI is similar to MPS IH, except intelligence is normal.


any of a group of genetically determined disorders due to a defect in glycosaminoglycan (GAG) metabolism, marked by skeletal changes, mental retardation and visceral involvement; abbreviated MPS. Achondroplastic dwarfism in cattle may be a defect of this type.

mucopolysaccharidosis I
caused by an inherited deficiency of α-l-iduronidase with increased urinary excretion of dermatan sulfate and heparan sulfate. Affected dogs and cats show facial dysmorphia, stunted growth, corneal clouding, lameness and granulation of leukocytes. Called also Hurler's syndrome.
mucopolysaccharidosis VI
caused by an inherited deficiency of arylsulfatase B with increased urinary excretion of dermatan sulfate. Affected Siamese cats show facial dysmorphia, corneal clouding, granulation of leukocytes, posterior paresis, and skin nodules. Called also Maroteaux-Lamy syndrome.
mucopolysaccharidosis type VII
caused by a deficiency of β-glucaronidase. Affected dogs have facial dysmorphism and corneal edema.
References in periodicals archive ?
Contract notice: Alimentary tract and metabolism of medicines - enzyme treatment of mucopolysaccharidosis vi (galsulfase).
Contract award: delivery galsulfase drug treatment program mucopolysaccharidosis vi teaching hospital no.

Full browser ?