Effects of X-monosomy and X-linked imprinting on superior temporal gyrus morphology in Turner syndrome
Effects of X-monosomy and X-linked imprinting on superior temporal gyrus morphology in Turner syndrome
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DOI：
10.1016/s0006-3223(03)00289-0
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发表时间：
2003-09-15
影响因子：
10.6
通讯作者：
Reiss, AL
中科院分区：
文献类型：
--
作者：
Kesler, SR;Blasey, CM;Reiss, AL
背景：Turner综合征(TS)是由完全或部分单体X引起的。TS的认知表型包括视觉空间功能缺陷的语言技能的保存。方法：对30例女性TS患者(平均年龄=14.73±6.41岁；范围=7.56~33.30岁)和30例年龄匹配的对照组(平均年龄=14.63±5.90岁；范围=6.35~32.65岁)的颞上回(STG)进行了测量。尽管TS受试者的左侧脑白质体积增加，但两组间的整体左侧STG体积并无显著差异。与具有父系X染色体(XP)的受试者和对照受试者相比，具有母系X染色体(XM)的TS亚组显示出更多的STG体积异常。XM和对照组受试者STG体积的差异涉及白质和灰质。结论：这些发现表明X单体和X连锁印记可能通过破坏神经修剪机制对STG的发育产生负面影响。
Background: Turner syndrome (TS) results from complete or partial monosomy X. The cognitive phenotype of TS involves preservation of verbal skills with visuospatial functioning deficits. The superior temporal gyrus (STG), which is involved in language capacities, has not been investigated in TS.Methods: The STG was measured in 30 female subjects (mean age=14.73+/-6.41; range=7.56-33.30) with TS and 30 age-matched control subjects (mean age=14.63+/-5.90; range=6.35-32.65) using volumetric magnetic resonance imaging analyses.Results: Right STG, including both gray and white matter volumes, was significantly larger in TS compared with control subjects. Overall left STG volume was not significantly different between groups, although left white matter volume was increased in the TS subjects. The TS subgroup with a maternally derived X chromosome (Xm) demonstrated more aberrant STG volumes compared with subjects with a paternally (Xp) derived X and control subjects. The difference in STG volumes between Xm and control subjects involved both white and gray matter. The Xm subjects differed from Xp subjects only in terms of gray matter.Conclusions: These findings suggest that X-monosomy and X-linked imprinting negatively affect STG development, possibly by disrupting neural pruning mechanisms.