ITPR1 antibodies

Indication

In case of suspected autoimmune encephalitis /autoimmune cerebellar ataxia.

Method

Indirect immunofluorescence (IIF)

Response

Results are reported as negative or positive. Positive results are reported with a titer.

Interpretation

Presence of autoantibodies to the protein inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) have been reported in cases of autoimmune cerebellar ataxia.

The inositol 1,4,5-trisphosphate receptors are localized to intracellular membranes, such as the endoplasmic reticulum. Inositol 1,4,5-trisphosphate receptors play an important role in intracellular Ca2 + signaling in a variety of cell types.

The presence of ITPR1 autoantibodies should be considered in the differential diagnosis of patients with subacute cerebellar ataxia of unknown cause.

The antigen has been defined as an intracellular antigen.

Generally, treatment with immunotherapy is more effective against auto-antibodies directed to extracellular antigens than with auto-antibodies against intracellular antigens.

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References

Sven Jarius, et.al., Antibodies to the inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) in cerebellar ataxia, Journal of Neuroinflammation, 2014, 11:206. PMID: 25498830

20 years experience