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Mucopolysaccharides

Glycosaminoglycans; GAG

 

Mucopolysaccharides are long chains of sugar molecules that are found throughout the body, often in mucus and in fluid around the joints. They are more commonly called glycosaminoglycans.

Information

 

When the body cannot break down mucopolysaccharides, a condition called mucopolysaccharidoses (MPS) occurs. MPS refers to a group of inherited disorders of metabolism. People with MPS do not have any, or enough, of a substance (enzyme) needed to break down the sugar molecule chains.

Forms of MPS include:

  • Hunter syndrome
  • Hurler syndrome
  • Sanfilippo syndrome
  • Scheie syndrome
  • Morquio syndrome

These conditions cause mucopolysaccharides to build up in body tissues. This can damage organs, including the heart. Symptoms can range from mild to severe, and may include:

  • Abnormal features of the face
  • Bone, cartilage, and connective tissue deformities
  • Mental disability

 

 

References

Pyeritz RE. Inherited diseases of connective tissue. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 260.

Spranger JW. Mucopolysaccharidoses. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 88.

Text only

 

        Tests for Mucopolysaccharides

         
         

        Review Date: 5/1/2017

        Reviewed By: Anna C. Edens Hurst, MD, MS, Assistant Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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