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Open Access Research

Differential expression of major histocompatibility complex class I in developmental glioneuronal lesions

Avanita S Prabowo1, Anand M Iyer1, Jasper J Anink1, Wim GM Spliet2, Peter C van Rijen2 and Eleonora Aronica134*

Author Affiliations

1 Department of (Neuro)Pathology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands

2 Departments of Pathology and Neurosurgery /Rudolf Magnus Institute for Neuroscience, University Medical Center Utrecht, Utrecht, The Netherlands

3 SEIN – Stichting Epilepsie Instellingen Nederland, Heemstede, The Netherlands

4 Swammerdam Institute for Life Sciences, Center for Neuroscience, University of Amsterdam, Amsterdam, The Netherlands

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Journal of Neuroinflammation 2013, 10:12  doi:10.1186/1742-2094-10-12

Published: 24 January 2013

Abstract

Purpose

The expression of the major histocompatibility complex class I (MHC-I) in the brain has received considerable interest not only because of its fundamental role in the immune system, but also for its non-immune functions in the context of activity-dependent brain development and plasticity.

Methods

In the present study we evaluated the expression and cellular pattern of MHC-I in focal glioneuronal lesions associated with intractable epilepsy. MHC-I expression was studied in epilepsy surgery cases with focal cortical dysplasia (FCD I, n = 6; FCD IIa, n = 6 and FCD IIb, n = 15), tuberous sclerosis complex (TSC, cortical tubers; n = 6) or ganglioglioma (GG; n = 15) using immunocytochemistry. Evaluation of T lymphocytes with granzyme-B+ granules and albumin immunoreactivity was also performed.

Results

All lesions were characterized by MHC-I expression in blood vessels. Expression in both endothelial and microglial cells as well as in neurons (dysmorphic/dysplastic neurons) was observed in FCD II, TSC and GG cases. We observed perivascular and parenchymal T lymphocytes (CD8+, T-cytotoxic) with granzyme-B+ granules in FCD IIb and TSC specimens. Albumin extravasation, with uptake in astrocytes, was observed in FCD IIb and GG cases.

Conclusions

Our findings indicate a prominent upregulation of MHC-I as part of the immune response occurring in epileptogenic glioneuronal lesions. In particular, the induction of MHC-I in neuronal cells appears to be a feature of type II FCD, TSC and GG and may represent an important accompanying event of the immune response, associated with blood–brain barrier dysfunction, in these developmental lesions.

Keywords:
Focal cortical dysplasia; Ganglioglioma; Cortical tubers; Neurons; Microglia; Major histocompatibility complex (MHC) class I