Tilson M D
Arch Surg. 1982 Sep;117(9):1212-3. doi: 10.1001/archsurg.1982.01380330070017.
The spontaneously aneurysm-prone Blotchy mouse has a mutation on the X chromosome resulting in low hepatic copper levels; and copper is an essential cofactor for lysyl oxidase, which catalyzes reactions leading to the cross-linking of collagen and elastin. Population characteristics and family histories of patients with aneurysms suggest that aneurysmal disease may also be sex linked in man. Hepatic copper levels were determined in 13 patients who died with abdominal aortic aneurysms and in 13 control patients selected on the criterion of severe atherosclerotic occlusive disease of the abdominal aorta. Excluding two patients with severe liver disease, the tissue copper level in the patients with aneurysms was only 26% of the control level. The results suggest that additional studies of the biologic markers for aneurysm formation in the Blotchy mouse should be carried out prospectively in human subjects.
自发易患动脉瘤的斑点小鼠在X染色体上发生突变，导致肝脏铜水平降低；而铜是赖氨酰氧化酶的必需辅因子，该酶催化导致胶原蛋白和弹性蛋白交联的反应。动脉瘤患者的人群特征和家族病史表明，动脉瘤疾病在人类中可能也与性别有关。对13例死于腹主动脉瘤的患者以及13例以腹主动脉严重动脉粥样硬化闭塞性疾病为标准选取的对照患者测定了肝脏铜水平。排除两名患有严重肝病的患者后，动脉瘤患者的组织铜水平仅为对照水平的26%。结果表明，应前瞻性地在人类受试者中对斑点小鼠动脉瘤形成的生物标志物进行更多研究。
