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25207 Alpha-Galactosidase, Serum (AGAS)

Alpha-Galactosidase, Serum (AGAS)
Test Code: AGASSO
Synonyms/Keywords
a-Gal A
Alpha Galactosidase
Anderson Fabry Disease
Ceramide Trihexosidase
Fabry Disease
Fabry's Disease
Galactosidase, Alpha
GLA Deficiency
Anderson-Fabry Disease
Useful For

Diagnosis of Fabry disease in male patients

Preferred screening test (serum) for Fabry disease

This test is not useful for patients undergoing a work up for a meat or meat-derived product allergy.

 

Genetics Test Information:

Serum is the preferred screening specimen for Fabry disease.

Enzyme testing is useful in identifying affected male patients.

Specimen Requirements
Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
Serum​ ​Red Top Tube (RTT) ​Serum Separator Tube (SST) ​2 mL ​0.2 mL
Collection Processing Instructions
Sex of patient is required for interpretation of results. 
Specimen Stability Information
Specimen Type Temperature Time
​Serum ​ ​Frozen (preferred) ​14 days
​Refrigerated ​24 hours
Interference

​Individuals with pseudodeficiency allelic variants can show reduced alpha-galactosidase A enzyme activity with this assay.

Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
​Mayo Clinic Laboratories ​Varies ​8 to 15 days
Fluorometric
Reference Lab
Test Information

Ordering Guidance:

If testing needed for assessment of meat or meat-derived product allergy, order either ALGAL / Galactose-Alpha-1,3-Galactose (Alpha-Gal), IgE, Serum or APGAL / Galactose-Alpha-1,3-Galactose (Alpha-Gal) Mammalian Meat Allergy Profile, Serum.

Carrier detection using enzyme levels is unreliable for female patients as results may be within the normal values. Order FABRZ / Fabry Disease, Full Gene Analysis, Varies for testing carrier status.

Additional Testing Requirements:

Urine sediment analysis (CTSU / Ceramide Trihexosides and Sulfatides, Random, Urine) for the accumulating trihexoside substrate and measurement of globotriaosylsphingosine (LGB3S / Gobotriaosylsphingosine, Serum) are also recommended.

Reference Range Information
Performing Location Reference Range
​Mayo Clinic Laboratories

​0.074-0.457 U/L

Note: Results from this assay are not useful for carrier determination. Carriers usually have levels in the normal range.

Interpretation

Deficiency (<0.016 U/L) of alpha-galactosidase in properly submitted specimens is diagnostic for Fabry disease in male patients. If concerned about specimen integrity, recheck using leukocyte testing (AGAW / Alpha-Galactosidase, Leukocytes).

Outreach CPTs
CPT Modifier
(if needed)
Quantity Description Comments
82657​
Synonyms/Keywords
a-Gal A
Alpha Galactosidase
Anderson Fabry Disease
Ceramide Trihexosidase
Fabry Disease
Fabry's Disease
Galactosidase, Alpha
GLA Deficiency
Anderson-Fabry Disease
Ordering Applications
Ordering Application Description
​Centricity ​Alpha-Galactosidase, S (AGAS)
​Cerner ​None
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
Serum​ ​Red Top Tube (RTT) ​Serum Separator Tube (SST) ​2 mL ​0.2 mL
Collection Processing
Sex of patient is required for interpretation of results. 
Specimen Stability Information
Specimen Type Temperature Time
​Serum ​ ​Frozen (preferred) ​14 days
​Refrigerated ​24 hours
Interference

​Individuals with pseudodeficiency allelic variants can show reduced alpha-galactosidase A enzyme activity with this assay.

Useful For

Diagnosis of Fabry disease in male patients

Preferred screening test (serum) for Fabry disease

This test is not useful for patients undergoing a work up for a meat or meat-derived product allergy.

 

Genetics Test Information:

Serum is the preferred screening specimen for Fabry disease.

Enzyme testing is useful in identifying affected male patients.

Reference Range Information
Performing Location Reference Range
​Mayo Clinic Laboratories

​0.074-0.457 U/L

Note: Results from this assay are not useful for carrier determination. Carriers usually have levels in the normal range.

Interpretation

Deficiency (<0.016 U/L) of alpha-galactosidase in properly submitted specimens is diagnostic for Fabry disease in male patients. If concerned about specimen integrity, recheck using leukocyte testing (AGAW / Alpha-Galactosidase, Leukocytes).

For more information visit:
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
​Mayo Clinic Laboratories ​Varies ​8 to 15 days
Fluorometric
Reference Lab
For billing questions, see Contacts
Outreach CPTs
CPT Modifier
(if needed)
Quantity Description Comments
82657​
For most current information refer to the Marshfield Laboratory online reference manual.