1977
DOI: 10.1126/science.144321
|View full text |Cite
|
Sign up to set email alerts
|

Mucopolysaccharidosis in a Cat with Arylsulfatase B Deficiency: A Model of Maroteaux-Lamy Syndrome

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 … Show more

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
1
1
1

Citation Types

1
65
0

Year Published

1987
1987
2012
2012

Publication Types

Select...
5
4

Relationship

2
7

Authors

Journals

citations
Cited by 109 publications
(66 citation statements)
references
References 18 publications
1
65
0
Order By: Relevance
“…Several naturally occurring MPS animal models exist, [15][16][17][18] providing an excellent source of materials to study this important disease mechanism. The data presented, along with our previous studies, 2,3 show that a central consequence of GAG storage in MPS animals is inflammation.…”
Section: Discussionmentioning
confidence: 99%
“…Several naturally occurring MPS animal models exist, [15][16][17][18] providing an excellent source of materials to study this important disease mechanism. The data presented, along with our previous studies, 2,3 show that a central consequence of GAG storage in MPS animals is inflammation.…”
Section: Discussionmentioning
confidence: 99%
“…The MPS VI cat and rat models, and MPS VII dog model have been previously described (2)(3)(4). Affected and control animals were raised under National Institutes of Health and USDA guidelines for the care and use of animals in research.…”
Section: Animalsmentioning
confidence: 99%
“…There are numerous MPS animal models available for study, either naturally occurring or generated through targeted disruption of the corresponding mouse gene (e.g., [2][3][4][5]. These animal models have biochemical and pathologic features that are similar to their human counterparts, and their availability has facilitated the evaluation of various therapeutic approaches and a better understanding of the disease pathogenesis.…”
mentioning
confidence: 99%
“…This disease has been described in humans, dogs, mice, and a single cat from Switzerland. [2][3][4][5][6][7][8]12 Canine and murine animal models have been used to investigate enzyme replacement, bone marrow transplantation, and gene therapy as possible treatments of the disease. 1,[9][10][11] Here, we describe the clinical and postmortem findings of MPS VII in a cat.…”
mentioning
confidence: 99%