Mirabella Federica, Gulisano Mariangela, Capelli Mara, Lauretta Giovanni, Cirnigliaro Matilde, Palmucci Stefano, Stella Michele, Barbagallo Davide, Di Pietro Cinzia, Purrello Michele, Ragusa Marco, Rizzo Renata
Section of Biology and Genetics Giovanni Sichel, Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy.
Section of Child and Adolescent Psychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
Front Mol Neurosci. 2021 Jan 5;13:608355. doi: 10.3389/fnmol.2020.608355. eCollection 2020.
Due to its rarity, coupled to a multifactorial and very heterogeneous nature, the molecular etiology of Arnold-Chiari (AC) syndrome remains almost totally unknown. Its relationship with other neuropsychiatric disorders such as Tourette syndrome (TS) is also undetermined. The rare comorbid status between both disorders (ACTS) complicates the framework of diagnosis and negatively affects the patients' quality of life. In this exploratory study, we aimed to identify serum microRNA expression profiles as molecular fingerprints for AC, TS, and ACTS, by using a high-throughput approach. For this aim, 10 AC patients, 11 ACTS patients, 6 TS patients, and 8 unaffected controls (NC) were recruited. Nine miRNAs resulted significantly differentially expressed (DE): let-7b-5p (upregulated in ACTS vs. TS); miR-21-5p (upregulated in ACTS vs. AC; downregulated in AC vs. TS); miR-23a-3p (upregulated in TS vs. NCs; downregulated in AC vs. TS); miR-25-3p (upregulated in AC vs. TS and NCs; downregulated in ACTS vs. AC); miR-93-5p (upregulated in AC vs. TS); miR-130a-3p (downregulated in ACTS and TS vs. NCs); miR-144-3p (downregulated in ACTS vs. AC; upregulated in AC vs. TS); miR-222-3p (upregulated in ACTS vs. NCs); miR-451a (upregulated in AC vs. TS and NCs; in ACTS vs. NCs). Altered expression of miRNAs was statistically correlated to neuroimaging and neuropsychological anomalies. Furthermore, computational analyses indicated that DE miRNAs are involved in AC and TS pathomechanisms. Finally, we propose the dysregulation of the miRNA set as a potential molecular tool for supporting the current diagnosis of AC, TS, and ACTS by using liquid biopsies, in an unbiased and non-invasive way.
由于阿诺德 - 基亚里(AC)综合征罕见,再加上其多因素且高度异质性的特点,其分子病因几乎完全未知。它与其他神经精神疾病如妥瑞氏症(TS)的关系也尚未确定。这两种疾病(ACTS)之间罕见的共病状态使诊断框架变得复杂,并对患者的生活质量产生负面影响。在这项探索性研究中,我们旨在通过高通量方法确定血清微小RNA表达谱,作为AC、TS和ACTS的分子指纹。为此,招募了10名AC患者、11名ACTS患者、6名TS患者和8名未受影响的对照(NC)。9种微小RNA结果显示有显著差异表达(DE):let - 7b - 5p(在ACTS与TS中上调);miR - 21 - 5p(在ACTS与AC中上调;在AC与TS中下调);miR - 23a - 3p(在TS与NC中上调;在AC与TS中下调);miR - 25 - 3p(在AC与TS和NC中上调;在ACTS与AC中下调);miR - 93 - 5p(在AC与TS中上调);miR - 130a - 3p(在ACTS和TS与NC中下调);miR - 144 - 3p(在ACTS与AC中下调;在AC与TS中上调);miR - 222 - 3p(在ACTS与NC中上调);miR - 451a(在AC与TS和NC中上调;在ACTS与NC中)。微小RNA的表达改变与神经影像学和神经心理学异常在统计学上相关。此外,计算分析表明差异表达的微小RNA参与了AC和TS的发病机制。最后,我们提出微小RNA集的失调作为一种潜在的分子工具,通过液体活检以无偏见和非侵入性的方式支持当前对AC、TS和ACTS的诊断。