Mucopolysaccharides

Glycosaminoglycans; GAG

Definition

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 sugar molecule chains.

Forms of MPS include:

  • MPS I (Hurler syndrome; Hurler-Scheie syndrome; Scheie syndrome)
  • MPS II (Hunter syndrome)
  • MPS III (Sanfilippo syndrome)
  • MPS IV (Morquio syndrome)

References

Kumar V, Abbas AK, Aster JC. Genetic disorders. In: Kumar V, Abbas AK, Aster JC, eds. Robbins & Cotran Pathologic Basis of Disease. 10th ed. Philadelphia, PA: Elsevier; 2021:chap 5.

Lampe C. Mucopolysaccharidoses. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 109.

Pyeritz RE. Inherited diseases of connective tissue. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 239.

Turnpenny PD, Ellard S, Cleaver R. Inborn errors of metabolism. In: Turnpenny PD, Ellard S, Cleaver R, eds. Emery's Elements of Medical Genetics and Genomics. 16th ed. Philadelphia, PA: Elsevier; 2022:chap 18.

GO

Review Date: 4/8/2025

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

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