The activity of a-L-iduronidase was found to be deficient in extracts prepared from livers, cultured fibroblasts and urine of patients with Hurler's syndrome. Extracts from livers and fibroblasts of Hunter's and Sanfilippo's syndromes did not show such a deficiency. There was a diminution of activity in extracts of "I-cell" fibroblasts. a-L-Iduronidase is believed to be the underlying enzymic defect of Hurler's disease.
|Original language||English (US)|
|Number of pages||6|
|Journal||Biochemical and Biophysical Research Communications|
|State||Published - May 26 1972|
ASJC Scopus subject areas
- Molecular Biology
- Cell Biology