Mucopolysaccharides Screen (MPS), Urine
Glycosaminoglycans, MPS Screen, Mucopolysaccharides, Urine Quantitative, MAYO: MPSSC, EPIC: LAB6108, SOFT: XAMUC
Specimen Collection Criteria
Collect: 3.0 mL random urine specimen in a screw-capped container (preferred) or other sterile urine container. An early morning (first void) specimen is preferred. (Min: 1.5 mL)
Critical frozen. Freeze specimen immediately after collection.
Physician Office/Drawsite Specimen Preparation
Critical frozen. Maintain frozen (-20°C/-4°F or below) prior to transport.
Preparation for Courier Transport
Transport: 3.0 mL random urine specimen, frozen (-20°C/-4°F or below). (Min: 1.5 mL)
- Specimens not collected and processed as indicated.
Specimen Stability for Testing:
Room Temperature (20-26°C or 68-78.8°F): Unacceptable
Refrigerated (2-8°C or 36-46°F): Unacceptable
Frozen (-20°C/-4°F or below): 365 days
Specimen Storage in Department Prior to Disposal:
Specimen retention time is determined by the policy of the reference laboratory. Contact the Sendout Laboratory with any questions.
Sent to Mayo Medical Laboratories, Rochester, MN.
Monday - Friday.
Results available in 11-19 days.
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS), Spectrophotometry.
Glycosamineoglycan (GAG), also known as mucopolysaccharides, are sulfated polysaccharides that contain repetitive disaccharide unites attached to a protein core.
Mucopolysaccharidoses (MPS) are disorders caused by defects of the lysosomal enzymes involved in the degradation of GAG. These defects cause the accumulation of partially degraded GAG, particularly in lysosomes of mesenchymal and parenchymal tissues and excessive secretion of these accumulated compounds in tissue.
An abnormally elevated excretion of GAG is characteristic of MPS.
The mucopolysaccharidoses are a group of disorders including Hurler/Scheie, Hunter, Sanfilippo, Morquio, Maroteaux-Lamy, and Sly asssociated with coarse features, short stature, skeletal abnormalities with or without corneal clouding, cardiac complications, hepatosplenomegaly, and mental retardation. There is widespread lysosomal storage of mucopolysaccharides and increased excretion of dermatan, heparin, keratin, and/or chondroitin sulfate - collectively designated as glycosaminglycans (GAG).
MAYO: MPSSC, EPIC: LAB6108, SOFT: XAMUC