Stam Anine H, van den Maagdenberg Arn M J M, Haan Joost, Terwindt Gisela M, Ferrari Michel D
Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.
Curr Opin Neurol. 2008 Jun;21(3):288-93. doi: 10.1097/WCO.0b013e3282fd171a.
To highlight recent genetic findings in migraine and discuss, new mutations in hemiplegic migraine genes in familial and sporadic cases and relevant candidate gene association studies. Special attention will be given to comorbid diseases of migraine.
Familial hemiplegic migraine (FHM) is genetically heterogeneous with mutations in the CACNA1A (FHM1), ATP1A2 (FHM2) and SCN1A (FHM3) genes. Nineteen novel ATP1A2 mutations were identified last year, eleven of them in FHM2 families. A systematic genetic analysis of patients with sporadic hemiplegic migraine revealed five mutations in this gene, which has implications for genetic counselling. The identification of a second FHM3 SCN1A mutation definitely established SCN1A as a migraine gene. The identification of TREX1 mutations in families with retinal vasculopathy and associated diseases such as migraine may provide new insights in migraine pathophysiology.
Many novel ATP1A2 mutations were identified in patients with familial and sporadic hemiplegic migraine. In sporadic patients, ATP1A2 screening has the highest chance of finding a causal mutation. A second FHM3 mutation definitely established the epilepsy SCN1A gene as a migraine gene. The discovery of genes in monogenic diseases in which migraine is prominent may lead to new insights in the molecular pathways involved in migraine pathophysiology.
强调偏头痛近期的遗传学发现,并讨论家族性和散发性病例中偏瘫性偏头痛基因的新突变以及相关候选基因关联研究。将特别关注偏头痛的合并症。
家族性偏瘫性偏头痛(FHM)在基因上具有异质性,与CACNA1A(FHM1)、ATP1A2(FHM2)和SCN1A(FHM3)基因的突变有关。去年鉴定出19种新的ATP1A2突变,其中11种存在于FHM2家族中。对散发性偏瘫性偏头痛患者进行的系统遗传学分析在该基因中发现了5种突变,这对遗传咨询具有重要意义。第二个FHM3 SCN1A突变的鉴定明确将SCN1A确定为偏头痛基因。在患有视网膜血管病变及偏头痛等相关疾病的家族中发现TREX1突变,可能为偏头痛的病理生理学提供新的见解。
在家族性和散发性偏瘫性偏头痛患者中鉴定出许多新的ATP1A2突变。在散发性患者中,ATP1A2筛查发现致病突变的可能性最高。第二个FHM3突变明确将癫痫SCN1A基因确定为偏头痛基因。在偏头痛突出的单基因疾病中发现基因,可能会为偏头痛病理生理学涉及的分子途径带来新的见解。