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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Nuclear abnormalities in vascular myocytes in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
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Nuclear abnormalities in vascular myocytes in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

机译：脑常染色体显性动脉患者血管肌细胞的核异常与皮下梗死和白细胞病（Cadasil）

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Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a stroke and dementia syndrome with degeneration and loss of vascular smooth muscle cells (VSMCs). The disease is due to mutations in NOTCH3 playing an important role in VSMC differentiation, proliferation and apoptosis. Searching for a possible cause of VSMC dysfunction in CADASIL, we investigated morphology and proliferative activity the affected myocytes. In material from autopsy brains and skin-muscle biopsies of patients with CADASIL diagnosis, assessment of VSMCs in arterial vessels at the level of light and electron microscopy was performed. Proliferative activity of VSMCs was evaluated in immune reactions to proliferative markers: proliferating cell nuclear antigen, and cyclins B1 and D. In CADASIL, abnormal morphology of VSMC nuclei was observed in 18.1%, 11.5%, and 6.9% of the cerebral, skin, and skeletal muscle vessels, respectively. The affected myocytes showed variability in nuclear size, irregularity in nuclear shape, and abnormal chromatin appearance. Frequently, double nuclei of equal size or micronuclei were observed. Sometimes, even multinuclear myocytes were found. In some of the nuclei immune reactions to the examined proliferative markers were positive. Aberrant structure and number of VSCM nuclei, and their immunoreactivity to proliferative markers suggest mitotic instability of vascular myocytes in CADASIL. We speculate that mutant NOTCH3 which is unable to control properly VSMC proliferation, and may be responsible for their premature or inappropriate entry into mitosis, irreversible arrest of the cell cycle, senescence or degeneration and loss.
机译：脑常染色体显性动脉瘤和白细胞病（Cadasil）是一种卒中和痴呆综合征，具有退化和血管平滑肌细胞（VSMC）的丧失。该病是由于Notch3中的突变在VSMC分化，增殖和凋亡中发挥着重要作用。在Cadasil中寻找可能的VSMC功能障碍的可能原因，我们研究了受影响的肌细胞的形态和增殖活性。在尸检大脑和皮肤肌肉活检的材料中，进行了患者的患者，在光线和电子显微镜水平下进行动脉血管中的VSMC。在增殖标记的免疫反应中评价VSMC的增殖活性：增殖细胞核抗原，以及在Cadasil中，在18.1％，11.5％和6.9％的脑，皮肤，皮肤，皮肤，皮肤，皮肤，皮肤，皮肤的异常形态，和骨骼肌血管分别。受影响的肌细胞显示核大小的可变性，核形状的不规则性和染色质异常。通常，观察到相同大小或微核的双核。有时，甚至发现了甚至多核肌细胞。在一些核免疫反应中，对所检测的增殖标记物是阳性的。异常结构和VSCM核的数量，以及它们对增殖标记的免疫反应性表明Cadasil中血管肌细胞的有丝分裂不稳定性。我们推测突变体Notch3无法控制持续扩散的突变体，并且可能对其过早或不恰当的进入有丝分裂，细胞周期，衰老或变性和损失的不可逆转逮捕。

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