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Mucopolysaccharides
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Mucopolysaccharides

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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.

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    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
    • Intellectual disability

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References

National Institute of Neurological Disorders and Stroke. Mucolipidoses Fact Sheet. Bethesda, MD: Office of Communications and Public Liaison; February 13, 2007. Publication No. 03-5115.

Wraith JE. Mucopolysaccharidoses and oligosaccharidoses. In: Saudubray J-M, van den Berghe G, Walter JH, eds. Inborn Metabolic Diseases: Diagnosis and Treatment. 5th ed. New York, NY: Springer; 2012:chap 40.

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Review Date: 5/7/2013  

Reviewed By: Chad Haldeman-Englert, MD, FACMG, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.

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