What is MPS IV?
MPS IV is a mucopolysaccharide disease known as Morquio or Morquio-Brailsford syndrome. MPS IV takes its name from Dr. Morquio, a pediatrician in Montevideo, Uruguay, who in 1929 described a family of four children affected by this condition. As the same condition was also described in the same year by Dr. Brailsford from Birmingham, England, it is sometimes known as Morquio-Brailsford syndrome. MPS IV has a wide range of symptoms that vary in severity and can be managed and treated with enzyme replacement therapies. There is no cure for MPS IV.
What Causes This Disease?
Mucopolysaccharides are chains of sugar molecules used to build connective tissues in the body.
“muco” refers to the thick jelly-like consistency of the molecules
“poly” means many
“saccharide” is a general term for a sugar molecule
The body constantly replaces used materials and breaks them down for disposal. MPS IV patients are missing an enzyme essential to breaking down the mucopolysaccharide keratan sulfate. These materials remain stored in the body’s cells, causing progressive damage. Babies may show little sign of the disease, but as cells sustain damage, symptoms start to appear.
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