Cavanagh K, Dunstan R W, Jones M Z
Am J Vet Res. 1982 Jun;43(6):1058-9.
Plasma alpha- and beta-mannosidase activities were measured in goats with beta-mannosidosis, a recently discovered, inherited deficiency of the glycohydrolase beta-D-mannosidase. Plasma beta-mannosidase activity in the affected animals was zero at 1 and 4 weeks after they were born. Enzyme values of age-matched, unaffected goats were between 66 and 222 nmol/hr/ml of plasma, alpha-Mannosidase activities were similar in both affected and unaffected animals. This investigation indicates that deficiency of plasma beta-mannosidases activity may be diagnostic of beta-mannosidosis.
对患有β-甘露糖苷贮积症(一种最近发现的、遗传性的糖水解酶β-D-甘露糖苷酶缺乏症)的山羊进行了血浆α-和β-甘露糖苷酶活性测定。患病动物出生后1周和4周时,血浆β-甘露糖苷酶活性为零。年龄匹配的未患病山羊的酶值在每毫升血浆66至222纳摩尔/小时之间,患病和未患病动物的α-甘露糖苷酶活性相似。这项研究表明,血浆β-甘露糖苷酶活性缺乏可能是β-甘露糖苷贮积症的诊断依据。