猫黏多糖贮积症 I 型模型的病理学
The pathology of the feline model of mucopolysaccharidosis I.
作者信息
Haskins M E, Aguirre G D, Jezyk P F, Desnick R J, Patterson D F
出版信息
Am J Pathol. 1983 Jul;112(1):27-36.
Abstract
Five cats with feline alpha-L-iduronidase-deficient mucopolysaccharidosis were studied. Membrane-bound cytoplasmic inclusions were present in central nervous system neurons, hepatocytes, chondrocytes, vascular and splenic smooth muscle cells, bone marrow leukocytes, and fibroblasts of the skin, eye, and cardiac valves. The lesions in these cats closely resemble those described in human patients with mucopolysaccharidosis I H (Hurler syndrome).
摘要
对五只患有猫α-L-艾杜糖苷酶缺乏性黏多糖贮积症的猫进行了研究。在中枢神经系统神经元、肝细胞、软骨细胞、血管和脾脏平滑肌细胞、骨髓白细胞以及皮肤、眼睛和心脏瓣膜的成纤维细胞中存在膜结合的细胞质内含物。这些猫的病变与人类黏多糖贮积症I H（Hurler综合征）患者中描述的病变非常相似。
Zotero 插件