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MPS IV Morquio

key information

source: MPS Society

year: N/A

summary/abstract:

What is MPS IV?

MPS IV, known as Morquio disease, is one of the mucopolysaccharide storage diseases. MPS IV disease was first identified by Dr Morquio in 1929 and includes 2 different types A and B.

What Causes MPS IV?

Mucopolysaccharides are long chains of sugar molecules used in the building of bones, cartilage, skin, tendons and many other tissues in the body. “Muco” refers to the thick jelly-like consistency of the sugar molecules, “poly” means many, and “saccharide” is a general term for the sugar part of the molecule. In the course of normal life there is a continuous recycling process of building new mucopolysaccharides and breaking down old ones. The breakdown and recycling process requires a series of special biochemical tools called enzymes.

People with MPS IV are either type A or B. Each type is missing or low in in a specific enzyme

·         MPS IVA is missing or low in N-acetyl-galactosamine 6-sulfatase

·         MPS IVB is missing or low in beta-galactosidase

 

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