Nyholt D R, Lea R A, Goadsby P J, Brimage P J, Griffiths L R
Genomics Research Centre, Griffith University--Gold Coast, Queensland, Australia.
Neurology. 1998 May;50(5):1428-32. doi: 10.1212/wnl.50.5.1428.
Migraine is a frequent familial disorder that, in common with most multifactorial disorders, has an unknown etiology. The authors identified several families with multiple individuals affected by typical migraine using a single set of diagnostic criteria and studied these families for cosegregation between the disorder and markers on chromosome 19, the location of a mutation that causes a rare form of familial hemiplegic migraine (FHM). One large tested family showed both cosegregation and significant allele sharing for markers situated within or adjacent to the FHM locus. Multipoint GENEHUNTER results indicated significant excess allele sharing across a 12.6-cM region containing the FHM Ca2+ channel gene, CACNL1A4 (maximum nonparametric linkage Z score = 6.64, p = 0.0026), with a maximum parametric lod score of 1.92 obtained for a (CAG)n triplet repeat polymorphism situated in exon 47 of this gene. The CAG expansion did not, however, appear to be the cause of migraine in this pedigree. Other tested families showed neither cosegregation nor excess allele sharing to chromosome 19 markers. HOMOG analysis indicated heterogeneity, generating a maximum HLOD score of 3.6. It was concluded that Chr19 mutations either in the CACNL1A4 gene or a closely linked gene are implicated in some pedigrees with familial typical migraine, and that the disorder is genetically heterogeneous.
偏头痛是一种常见的家族性疾病,与大多数多因素疾病一样,其病因不明。作者使用一套单一的诊断标准,识别出几个有多名个体受典型偏头痛影响的家族,并研究了这些家族中该疾病与19号染色体上标记之间的共分离情况,19号染色体上存在一种导致罕见形式的家族性偏瘫性偏头痛(FHM)的突变。一个经过检测的大家系显示,位于FHM基因座内或附近的标记存在共分离和显著的等位基因共享。多点GENEHUNTER分析结果表明,在一个包含FHM钙离子通道基因CACNL1A4的12.6厘摩区域内,等位基因共享显著过量(最大非参数连锁Z分数 = 6.64,p = 0.0026),对于位于该基因第47外显子的(CAG)n三联体重复多态性,获得的最大参数连锁lod分数为1.92。然而,在这个家系中,CAG扩增似乎不是偏头痛的病因。其他经过检测的家系既未显示与19号染色体标记的共分离,也未显示等位基因共享过量。HOMOG分析表明存在遗传异质性,产生的最大HLOD分数为3.6。研究得出结论,CACNL1A4基因或紧密连锁基因中的19号染色体突变与一些家族性典型偏头痛家系有关,并且该疾病在遗传上是异质性的。