Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome.
Science
; 198(4319): 834-6, 1977 Nov 25.
Article
em En
| MEDLINE
| ID: mdl-144321
ABSTRACT
A Siamese cat that presented clinical signs similar to those seen in humans with mucopolysaccharidoses was studied. The animal excreted increased amounts of polymeric glycosaminoglycans in the urine, consisting almost entirely of dermatan sulfate. Electron microscopy of circulating polymorphonuclear leukocytes revealed the presence of many membrane-bound lamellar inclusion bodies. Sulfate incorporation studies with cultured skin fibroblasts indicated defective glycosaminoglycan degradation. These cells showed a deficiency in arylsulfatase B activity. The disorder appears similar or identical to the Maroteaux-Lamy syndrome described in humans.
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Base de dados:
MEDLINE
Assunto principal:
Sulfatases
/
Doenças do Gato
/
Condro-4-Sulfatase
/
Mucopolissacaridoses
/
Mucopolissacaridose VI
/
Modelos Animais de Doenças
Tipo de estudo:
Prognostic_studies
Limite:
Animals
Idioma:
En
Ano de publicação:
1977
Tipo de documento:
Article