Test Catalog

Test ID: ARSB    
Arylsulfatase B, Fibroblasts

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 genetic test or proper submission of the test request

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


This test is not recommended for prenatal testing.


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 tests that are always performed, at an additional charge, with the initial tests.

Test IDReporting NameAvailable SeparatelyAlways Performed
FIBRFibroblast CultureYesYes
CRYOBCryopreserve for Biochem StudiesNoYes

Testing Algorithm Delineates situations 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 Library of PDFs including pertinent information and forms related to the test

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

ARSB: Colorimetric Enzyme Assay

CRYOB: Fibroblast Subculture Followed by Cryopreservation and Storage

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


Reporting Name Lists a shorter or abbreviated version of the Published Name for a test

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