What is MPS IX (Natowicz Syndrome)?
People with MPS IX are low in an enzyme called hyaluronidase which is essential in breaking down mucopolysaccharide hyaluronan. When hyaluronan is not completely broken down it remains stored in the body, and this casues the symptoms of MPS IX.
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
People with MPS IX are low in an enzyme called hyaluronidase which is essential in breaking down mucopolysaccharide hyaluronan. When hyaluronan is not completely broken down it remains stored in the body. The symptoms of MPS IX are a result of the build-up of hyaluronan in the tissues in the body. Babies may show little sign of the disease but as more and more cells build up with partially broken down mucopolysaccharides, symptoms start to appear.
To request more information about MPS and related LSD’s please contact us.
