ISSN:
1432-1076
Keywords:
Metachromatic leukodystrophy
;
Multiple sulfatase deficiencies
;
Urinary acid mucopolysaccharide
;
Heparan sulfate
;
Chondroitin sulfate A/C
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Urinary acid mucopolysaccharide (AMPS) excretion was investigated in a Japanese case with Multiple Sulfatase Deficiency (MSD) (Mucosulfatidosis). The patient excreted AMPS 4 to 5 times more (as carbazoluronic acid) than controls. The cellulose acetate gel electrophoresis clearly indicated two major AMPS which co-migrated with heparan sulfate and chondroitin sulfate A/C. Enzymic digestion with chondroitinase AC and ABC, and by testicular hyaluronidase plus amino sugar analysis also confirmed that our case excreted heparan sulfate and chondroitin sulfate A/C. These findings suggest that there are heterogeneities of urinary AMPS excretion among cases with MSD.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00442437
