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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.
Enzyme
Enzymes are complex proteins that cause a specific chemical change in all parts of the body. For example, they can help break down the foods we eat ...
Forms of MPS include:
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Hunter syndrome
Hunter syndrome
Hunter syndrome is a disease in which long chains of sugar molecules (glycosaminoglycans, formerly called mucopolysaccharides) are not broken down co...
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Hurler syndrome
Hurler syndrome
Hurler syndrome is a rare disease of metabolism in which a person cannot break down long chains of sugar molecules called glycosaminoglycans (formerl...
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Sanfilippo syndrome
Sanfilippo syndrome
Sanfilippo syndrome is metabolism disorder in which the body cannot properly break down long chains of sugar molecules. These chains of molecules ar...
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Scheie syndrome
Scheie syndrome
Scheie syndrome is metabolism disease in which the body cannot properly break down long chains of sugar molecules called glycosaminoglycans (formerly...
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Morquio syndrome
Morquio syndrome
Morquio syndrome is a disease of metabolism in which the body is missing or does not have enough of a substance needed to break down long chains of s...
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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
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Mental disability
Mental disability
Intellectual disability is a condition diagnosed before age 18 that includes below-average intellectual function and a lack of skills necessary for d...
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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.
Pyeritz RE. Inherited diseases of connective tissue. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 260.
Spranger J. Mucopolysaccharidoses. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics . 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 82.
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.
Review Date: 4/20/2015
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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
