{"title":"[粘液脂质病中唾液糖肽的分泌]。","authors":"M Lemonnier, D Lecat, R Bourrillon, P Maroteaux","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Mucolipidosis I involves a tremendous increase of the urinary excretion of sialoglycopeptides and sialyloligosaccharides. This enhancement is due to the excretion of O- and N-glycosidic peptides and oligosaccharides, which normal urine is devoid of as shown by the chemical composition and electrophoresis or thin-layer analysis. This finding is in agreement with the recent finding of an alpha-neuraminidase deficiency for this disease.</p>","PeriodicalId":10544,"journal":{"name":"Comptes rendus hebdomadaires des seances de l'Academie des sciences. Serie D: Sciences naturelles","volume":"287 4","pages":"353-6"},"PeriodicalIF":0.0000,"publicationDate":"1978-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Excretion of sialoglycopeptides in mucolipidosis I].\",\"authors\":\"M Lemonnier, D Lecat, R Bourrillon, P Maroteaux\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Mucolipidosis I involves a tremendous increase of the urinary excretion of sialoglycopeptides and sialyloligosaccharides. This enhancement is due to the excretion of O- and N-glycosidic peptides and oligosaccharides, which normal urine is devoid of as shown by the chemical composition and electrophoresis or thin-layer analysis. This finding is in agreement with the recent finding of an alpha-neuraminidase deficiency for this disease.</p>\",\"PeriodicalId\":10544,\"journal\":{\"name\":\"Comptes rendus hebdomadaires des seances de l'Academie des sciences. Serie D: Sciences naturelles\",\"volume\":\"287 4\",\"pages\":\"353-6\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1978-09-11\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Comptes rendus hebdomadaires des seances de l'Academie des sciences. Serie D: Sciences naturelles\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Comptes rendus hebdomadaires des seances de l'Academie des sciences. Serie D: Sciences naturelles","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
[Excretion of sialoglycopeptides in mucolipidosis I].
Mucolipidosis I involves a tremendous increase of the urinary excretion of sialoglycopeptides and sialyloligosaccharides. This enhancement is due to the excretion of O- and N-glycosidic peptides and oligosaccharides, which normal urine is devoid of as shown by the chemical composition and electrophoresis or thin-layer analysis. This finding is in agreement with the recent finding of an alpha-neuraminidase deficiency for this disease.