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The Journal of Headache and Pain

Official Journal of the "European Headache Federation" and of "Lifting The Burden - The Global Campaign against Headache"

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Open Access

EHMTI-0262. Dysregulation of inflammatory pathways in a familial hemiplegic migraine 1 mouse model after the induction of cortical spreading depression

  • B de Vries1,
  • EE Eising1,
  • R Shyti1,
  • LS Vijfhuizen1,
  • LAM Broos1,
  • PAC’t Hoen1,
  • MD Ferrari2,
  • EA Tolner1 and
  • AMJM van den Maagdenberg1
The Journal of Headache and Pain201415(Suppl 1):B5

Published: 18 September 2014


MigraineGene Expression ProfileMigraine With AuraInflammatory PathwayCortical Spreading Depression


Familial Hemiplegic Migraine type 1 (FHM1) is a rare monogenic subtype of migraine with aura caused by mutations in the CACNA1A gene. In FHM1 knock-in mouse models these mutations increase the susceptibility for cortical spreading depression (CSD): the underlying mechanism of the migraine aura.


To study the consequences of CSD in a migraine-relevant context, we measured cortical gene expression profiles in FHM1 and wild-type mice 24 hours after CSD induction.


Expression profiles were generated using deep-Serial Analysis of Gene Expression (SAGE) sequencing, a tag-based next-generation sequencing method for gene expression profiling. Relevant expression changes were validated by qPCR experiments.


Our data show that CSD induces differential expression of genes involved in inflammatory pathways in both the FHM1 and wild-type mice. However, we identified a gene set that is up-regulated upon CSD specifically in the FHM1 migraine mouse model. Genes from this gene set are involved in inflammatory and interferon-related signaling, and were often found up-regulated in immune-stimulated conditions.


Differential expression of genes involved in inflammatory pathways in the brain of FHM1 migraine mice compared to wild-type mice upon CSD, indicates that CSD affects the brain differently in a genetically predisposed animal which may help increase our understanding of migraine pathophysiology.

No conflict of interest

Authors’ Affiliations

Human Genetics, Leiden University Medical Center (LUMC), Leiden, Netherlands
Neurology, Leiden University Medical Center (LUMC), Leiden, Netherlands


© de Vries et al; licensee Springer. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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