Hurler syndrome (MPS I), Hunter syndrome (MPS II)
Hurler/Hunter Syndrome (MPS I/II): Urine Monitoring (Total GAGs, DS, HS)
Hurler/Hunter Syndrome (MPS I/II): Urine Monitoring (Total GAGs, DS, HS)
This test quantitatively measures total glycosaminoglycans, dermatan sulfate (uDS), and heparan sulfate (uHS) and can be used to monitor patients with Hurler syndrome (MPS I) or Hunter syndrome (MPS II).
Technical Information
Total glycosaminoglycans are analyzed by 1,9-dimethylene blue (DMB) colorimetric reaction that is measured by spectrophotometry. Dermatan sulfate and heparan sulfate are analyzed by liquid chromatography-tandem mass spectrometry (LC-MS/MS)
Specimen Requirements
Urine is the sample type for this test, and requires a minimum of 2 mL random catch urine.
Transport Instructions
The urine specimen should be frozen after collection and shipped frozen, preferably on dry ice.
Associated Tests
- Hunter Syndrome (MPS II): IDS Sequencing
- Hunter Syndrome (MPS II): Iduronate-2-Sulfatase Enzyme Analysis
- Hurler Syndrome (MPS I): Alpha-iduronidase Enzyme Analysis
- Hurler Syndrome (MPS I): IDUA Sequencing
- Mucopolysaccharidosis (MPS) Enzyme Panel
- Mucopolysaccharidosis (MPS) Enzyme Panel (DBS)
- Mucopolysaccharidosis (MPS) Urine Analysis (Total GAGs, DS, CS, KS, HS)
- Total Glycosaminoglycans (GAGs) Analysis
Connect With Our Experts
Call 1-800-473-9411 to speak with our team of laboratory genetic counselors for questions or additional information.
Robin Fletcher, MS, CGC
Falecia Thomas, MS, CGC
Alex Finley, MS, CGC