Leipold H W, Smith J E, Jolly R D, Eldridge F E
J Am Vet Med Assoc. 1979 Sep 1;175(5):457-9.
Mannosidosis, an inherited lysosomal storage disease, was diagnosed in two purebred Angus calves in the United States. Calf 1 was 5 months old and calf 2 was 7 months old when they were necropsied. Both calves had a history of progressive incoordination and ataxia. Gross postmortem findings included moderate hydrocephalus. Histologic examination revealed intracytoplasmic vacuolation of neurons throughout spinal cord and brain as well as in exocrine pancreatic cells, reticuloendothelial cells of the liver, and medullary sinusoids of lymph nodes. Biochemical study of 61 blood samples from the first calf's herd revealed bimodal distribution of enzyme activity for mannosidase. Two populations could be distinguished in that herd, those with normal mannosidase activity and those heterozygous for the disease.
甘露糖苷贮积症是一种遗传性溶酶体贮积病，在美国的两头纯种安格斯犊牛中被诊断出来。犊牛1在尸检时5个月大，犊牛2在尸检时7个月大。两头犊牛都有进行性共济失调和运动失调的病史。死后大体检查结果包括中度脑积水。组织学检查显示，整个脊髓、大脑以及外分泌胰腺细胞、肝脏的网状内皮细胞和淋巴结的髓窦中的神经元存在胞浆内空泡化。对第一头犊牛所在牛群的61份血样进行的生化研究显示，甘露糖苷酶的酶活性呈双峰分布。在那个牛群中可以区分出两个群体，即甘露糖苷酶活性正常的群体和该疾病的杂合子群体。
