Baker H J, Wood P A, Wenger D A, Walkley S U, Inui K, Kudoh T, Rattazzi M C, Riddle B L
Department of Comparative Medicine, Bowman Gray School of Medicine, Winston-Salem, NC.
Vet Pathol. 1987 Sep;24(5):386-91. doi: 10.1177/030098588702400504.
A 7-month-old Balinese cat with progressive neurological dysfunction had histopathological lesions of brain, liver, kidney, spleen, and lung consistent with a lysosomal storage disease. Ultrastructural examination revealed lysosomal hypertrophy with membranous inclusions. Hepatic sphingomyelin and cholesterol were elevated 10 times normal, and total phospholipids were increased 3.6 fold. Sphingomyelinase activity measured with 14C labeled sphingomyelin at pH 5.0 was virtually absent in brain and liver. Other lysosomal hydrolase activities were normal or elevated. Clinical, morphological, and biochemical findings suggest that this cat had sphingomyelin lipidosis similar to human Niemann-Pick disease type A, and that feline sphingomyelin lipidosis provides another model of human lysosomal storage disease.
一只患有进行性神经功能障碍的7个月大巴厘猫，其脑、肝、肾、脾和肺的组织病理学病变与溶酶体贮积病相符。超微结构检查显示溶酶体肥大并伴有膜性内含物。肝脏中的鞘磷脂和胆固醇升高至正常水平的10倍，总磷脂增加了3.6倍。在pH 5.0条件下用14C标记的鞘磷脂测量的鞘磷脂酶活性在脑和肝中几乎不存在。其他溶酶体水解酶活性正常或升高。临床、形态学和生化检查结果表明，这只猫患有与人类A型尼曼-匹克病相似的鞘磷脂沉积症，并且猫的鞘磷脂沉积症为人类溶酶体贮积病提供了另一种模型。
