Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A-and prosaposin-deficient mice
Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A-and prosaposin-deficient mice
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saposin A 和 prosaposin 缺陷小鼠睾丸中的赛米诺脂及其前体/降解产物半乳糖基烷基酰基甘油
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通讯作者：
H. Schachter
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作者：
P. Letts;R. Hunt;M. Shirley;L. Pinteric;H. Schachter
鞘脂激活蛋白（鞘脂激活蛋白A、B、C和D）来源于一种常见的前体蛋白（鞘脂激活蛋白原），并特异性激活短碳水化合物链糖脂的体内降解。鞘脂激活蛋白原缺乏（鞘脂激活蛋白原（cid：2）/（cid：2））的小鼠模型与多种糖脂升高的人类疾病非常相似。最近开发的saposin A（cid：2）/（cid：2）小鼠显示出慢性形式的球状细胞脑白质营养不良，确立了saposin A作为半乳糖神经酰胺酶的激活剂降解半乳糖神经酰胺的重要体内作用。精脂类是精子中的主要糖脂，其前体/降解产物半乳糖基烷基酰基甘油（GalEAG），
Sphingolipid activator proteins (saposins A, B, C, and D) are derived from a common precursor protein (pro-saposin) and specifically activate in vivo degradation of glycolipids with short carbohydrate chains. A mouse model of prosaposin deficiency (prosaposin (cid:2) / (cid:2) ) closely mimics the human disease with an elevation of multiple glycolipids. The recently developed saposin A (cid:2) / (cid:2) mice showed a chronic form of globoid cell leukodystrophy, establishing the essential in vivo role of saposin A as an activator for galactosylceramidase to degrade galactosylceramide. Seminolipid, the princi-pal glycolipid in spermatozoa, and its precursor/degradative product, galactosylalkylacylglycerol (GalEAG),