Morquio Syndrome
Alternate Names : Mucopolysaccharidosis Type IVA (Galactosamine-6-Sulfatase Deficiency), Mucopolysaccharidosis Type IVB (Beta Galactosidase Deficiency)
Definition
Morquio syndrome is an inherited disease belonging to the group of mucopolysaccharide storage diseases. Two forms are recognized, type A and type B.
Type A disease is characterized by the absence of the enzyme galactosamine-6-sulfatase, and the excretion of keratan sulfate in the urine. Type B disease results from deficiency of the enzyme beta galactosidase.
In both types there is accumulation in the body and brain of abnormally large amounts of a substance called mucopolysaccharide.
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