Brophy C M, Tilson J E, Braverman I M, Tilson M D
Department of Surgery, Yale University School of Medicine, New Haven, CT 06510.
J Vasc Surg. 1988 Jul;8(1):45-8.
The blotchy mouse has an X chromosome mutation affecting crosslinking of collagen and elastin, which results in aneurysmal dilatation of the aorta. The age of onset, patterns of distribution, and histologic features of these lesions have not been characterized in detail in previous studies. Male normal and blotchy mice 1 to 8 months of age were killed and latex was injected into the left ventricles to facilitate exposure, examination, histologic sampling, and photography of the aorta. Aneurysms were not detected in any normal animals but the affected animals had a progressive increase in the incidence of aneurysms with age, reaching 100% by 6 months. Most aneurysms occurred in the ascending aorta, with some also present in the descending thoracic and abdominal segments. Some animals had multiple aneurysms. Histologically the blotchy mice aortas exhibited disrupted elastic lamellae and thickening of the interlamellar spaces. These spaces contained conspicuously pleomorphic smooth muscle cells, confirmed by electron microscopy. These changes occurred as early as 21 days, when there was no gross evidence of aneurysmal development. Aortic aneurysms develop in blotchy mice in a consistent fashion, with characteristic gross and histologic changes. These animals provide a practical model for further studies of aneurysmal disease, including possible therapeutic interventions to prevent aneurysm development.
斑点小鼠存在一种影响胶原蛋白和弹性蛋白交联的X染色体突变，这会导致主动脉瘤样扩张。在以往的研究中，这些病变的发病年龄、分布模式和组织学特征尚未得到详细描述。处死1至8个月大的雄性正常小鼠和斑点小鼠，并将乳胶注入左心室，以便于暴露、检查、组织学取样和拍摄主动脉。在任何正常动物中均未检测到动脉瘤，但受影响的动物中动脉瘤的发生率随年龄增长而逐渐增加，到6个月时达到100%。大多数动脉瘤发生在升主动脉，部分也出现在胸降段和腹段。一些动物有多个动脉瘤。组织学上，斑点小鼠的主动脉表现为弹性板破裂和板层间隙增厚。这些间隙含有明显多形性的平滑肌细胞，电镜检查证实了这一点。这些变化早在21天时就已出现，此时尚无动脉瘤形成的明显肉眼证据。斑点小鼠的主动脉瘤以一致的方式发展，具有特征性的大体和组织学变化。这些动物为进一步研究动脉瘤疾病提供了一个实用模型，包括预防动脉瘤形成的可能治疗干预措施。
