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Test Catalog

Test ID: ARSB    
Arylsulfatase B, Fibroblasts

NY State Approved Indicates the status of NY State approval and if the test is orderable for NY State clients.


Useful For Suggests clinical disorders or settings where the test may be helpful

Diagnosis of mucopolysaccharidosis VI (Maroteaux-Lamy syndrome)

Genetics Test Information Provides information that may help with selection of the correct test or proper submission of the test request

Diagnostic testing for mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). Not recommended for carrier detection.


Arylsulfatase B deficiency results in mucopolysaccharidosis type VI (MPS VI), also called Maroteaux-Lamy syndrome.


Decreased or absent activity of arylsulfatase B in fibroblasts is diagnostic for MPS VI.


Arylsulfatase B activity may also be decreased in patients with multiple sulfatase deficiency and mucolipidosis II; however, the enzyme activity may not be as low as it is in patients with MPS VI.


This test is not indicated to determine carrier status for MPS VI.

Additional Tests Lists test(s) that are always performed, at an additional charge, with the initial test(s)

Test IDReporting NameAvailable SeparatelyAlways Performed
FIBRFibroblast CultureYesYes
CRYOBCryopreserve for Biochem StudiesNoYes

Testing Algorithm Delineates situation(s) when tests are added to the initial order. This includes reflex and additional tests.

When this test is ordered, a fibroblast culture and cryopreservation for biochemical studies will always be performed at an additional charge. However, for multiple lysosomal enzyme assays on a patient utilizing fibroblast culture, only 1 culture is required regardless of the number of enzyme assays ordered. If viable cells are not obtained within 10 days, client will be notified.

Special Instructions and Forms Describes specimen collection and preparation information, test algorithms, and other information pertinent to test. Also includes pertinent information and consent forms to be used when requesting a particular test

Method Name A short description of the method used to perform the test

ARSB: Colorimetric Enzyme Assay

FIBR: Cultivated from Biopsy as Monolayer

CRYOB: Fibroblast Subculture Followed by Cryopreservation and Storage

Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name

Arylsulfatase B, Fibroblasts

Aliases Lists additional common names for a test, as an aid in searching

Arylsulfatase B
Arylsulfatase B Deficiency
Maroteaux-Lamy Syndrome
Mucopolysaccharidosis Type VI
Mucopolysaccharidosis VI
N-Acetylgalactsamine-4-Sulfatase Deficiency