The Maroteaux-Lamy syndrome (Mucopolysaccharidosis VI) is characterized by excessive excretion in urine and deposition in tissue of dermatan sulfate. To investigate the enzymic basis of this disease [35S]chondroitin 4-sulfate was prepared utilizing a rat chondrosarcoma. Extracts of fibroblasts derived from patients with this syndrome showed a marked diminution of release of 35SO4 both from [35S]chondroitin 4-SO4 and from a heptasaccharide derived from this substance. Since there is a marked deficiency of arylsulfatase B activity in this disease, it is concluded that natural substrate of arylsulfatase B is N-acetylgalactosamine 4-SO4 in chondroitin 4-SO4 and dermatan sulfate.
|Original language||English (US)|
|Number of pages||8|
|Journal||Biochemical and Biophysical Research Communications|
|State||Published - Dec 23 1974|
ASJC Scopus subject areas
- Molecular Biology
- Cell Biology