Test Code APBTS Adaptor Protein 3 Beta2 (AP3B2) Antibody, Tissue Immunofluorescence Titer, Serum
Specimen Required
Only orderable as a reflex. For more information see:
-AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
-DYS2 / Dysautonomia, Autoimmune/Paraneoplastic Evaluation, Serum
-GID2 / Gastrointestinal Dysmotility, Autoimmune/Paraneoplastic Evaluation, Serum
-MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
Useful For
The differential diagnosis of patients presenting with mixed cerebellar and sensory ataxia and myeloneuropathy
Reporting an end titer result from serum specimens
Testing Algorithm
If the indirect immunofluorescence (IFA) pattern suggests AP3B2 (adaptor protein 3 beta2)-IgG, then AP3B2 antibody cell-binding assay (CBA) and this test will be performed at an additional charge.
Method Name
Only orderable as a reflex. For more information see:
-AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
-DYS2 / Dysautonomia, Autoimmune/Paraneoplastic Evaluation, Serum
-GID2 / Gastrointestinal Dysmotility, Autoimmune/Paraneoplastic Evaluation, Serum
-MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
Indirect Immunofluorescence Assay (IFA)
Reporting Name
AP3B2 IFA Titer, SSpecimen Type
SerumSpecimen Minimum Volume
See Specimen Required
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Refrigerated (preferred) | 28 days | |
Frozen | 28 days | ||
Ambient | 72 hours |
Reject Due To
Gross hemolysis | Reject |
Gross lipemia | Reject |
Gross icterus | Reject |
Clinical Information
AP3B2 (adaptor protein 3 beta2)-IgG is a marker of an autoimmune disorder unified by gait instability as the predominant neurologic presentation. Patients present with either cerebellar, dorsal column, or sensory neuronal dysfunction. Clinical improvement following treatment has been reported. AP3B2 autoimmunity appears rare, is accompanied by ataxia (sensory or cerebellar), and is potentially treatable.
Reference Values
Only orderable as a reflex. For more information see:
-AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
-DYS2 / Dysautonomia, Autoimmune/Paraneoplastic Evaluation, Serum
-GID2 / Gastrointestinal Dysmotility, Autoimmune/Paraneoplastic Evaluation, Serum
-MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
<1:240
Interpretation
A positive result supports a diagnosis of neurological autoimmunity. Neurological phenotypes encountered include cerebellar ataxia, spinocerebellar ataxia, myelopathy, sensory neuronopathy and autonomic neuropathy. Neurological stabilization or improvement may occur with immune therapy.
Cautions
A negative result does not exclude neurological autoimmunity or cancer.
Clinical Reference
Honorat JA, Lopez-Chiriboga AS, Kryzer, TJ, et al: Autoimmune gait disturbance accompanying adaptor protein-3B2-IgG. Neurology. 2019 Sep 3;93(10):e954-e963
Method Description
The patient’s specimen is tested by a standardized immunofluorescence assay that uses a composite frozen section of mouse cerebellum, kidney, and gut tissues. After incubation with sample and washing, fluorescein-conjugated goat-antihuman IgG is applied. Neuron-specific autoantibodies are identified by their characteristic fluorescence staining patterns. Specimens that are scored positive for any neuronal nuclear or cytoplasmic autoantibody are titrated to an endpoint. Interference by coexisting non-neuron-specific autoantibodies can usually be eliminated by serologic absorption.(Honorat JA, Komorowski L, Josephs KA, et al: IgLON5 antibody: neurological accompaniments and outcomes in 20 patients. Neurol Neuroimmunol Neuroinflamm 2017 Jul 18;4(5):e385. doi: 10.1212/NXI.0000000000000385)
Day(s) Performed
Monday through Sunday
Report Available
5 to 10 daysSpecimen Retention Time
28 daysPerforming Laboratory
Mayo Clinic Laboratories in RochesterTest Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
86256
LOINC Code Information
Test ID | Test Order Name | Order LOINC Value |
---|---|---|
APBTS | AP3B2 IFA Titer, S | 101908-2 |
Result ID | Test Result Name | Result LOINC Value |
---|---|---|
616109 | AP3B2 IFA Titer, S | 101908-2 |