Literature DB >> 144321

Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome.

P F Jezyk, M E Haskins, D F Patterson, W J Mellman, M Greenstein.   

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.

Entities:  

Mesh：

Substances：

Year:  1977        PMID： 144321     DOI： 10.1126/science.144321

Source DB:  PubMed          Journal:  Science        ISSN： 0036-8075            Impact factor:   47.728


  34 in total

1.  Enzyme replacement therapy from birth in a feline model of mucopolysaccharidosis type VI.

Authors:  A C Crawley; K H Niedzielski; E L Isaac; R C Davey; S Byers; J J Hopwood
Journal:  J Clin Invest       Date:  1997-02-15       Impact factor: 14.808

Review 2.  Lysosomal enzyme replacement therapies: Historical development, clinical outcomes, and future perspectives.

Authors:  Melani Solomon; Silvia Muro
Journal:  Adv Drug Deliv Rev       Date:  2017-05-11       Impact factor: 15.470

3.  Animal models for lysosomal storage diseases: a new case of feline mucopolysaccharidosis VI.

Authors:  P Di Natale; T Annella; A Daniele; G Spagnuolo; R Cerundolo; D de Caprariis; A E Gravino
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

4.  Enzyme replacement therapy in a feline model of Maroteaux-Lamy syndrome.

Authors:  A C Crawley; D A Brooks; V J Muller; B A Petersen; E L Isaac; J Bielicki; B M King; C D Boulter; A J Moore; N L Fazzalari; D S Anson; S Byers; J J Hopwood
Journal:  J Clin Invest       Date:  1996-04-15       Impact factor: 14.808

Review 5.  Murine mucopolysaccharidosis type VII: the impact of therapies on the clinical course and pathology in a murine model of lysosomal storage disease.

Authors:  C Vogler; M S Sands; N Galvin; B Levy; C Thorpe; J Barker; W S Sly
Journal:  J Inherit Metab Dis       Date:  1998-08       Impact factor: 4.982

Review 6.  Gene therapy for lysosomal storage diseases (LSDs) in large animal models.

Authors:  Mark Haskins
Journal:  ILAR J       Date:  2009

7.  A canine model of human alpha-L-iduronidase deficiency.

Authors:  E Spellacy; R M Shull; G Constantopoulos; E F Neufeld
Journal:  Proc Natl Acad Sci U S A       Date:  1983-10       Impact factor: 11.205

8.  Mechanism of glycosaminoglycan-mediated bone and joint disease: implications for the mucopolysaccharidoses and other connective tissue diseases.

Authors:  Calogera M Simonaro; Marina D'Angelo; Xingxuan He; Efrat Eliyahu; Nataly Shtraizent; Mark E Haskins; Edward H Schuchman
Journal:  Am J Pathol       Date:  2007-12-13       Impact factor: 4.307

9.  Arylsulfatase B-deficient mucopolysaccharidosis in rats.

Authors:  M Yoshida; J Noguchi; H Ikadai; M Takahashi; S Nagase
Journal:  J Clin Invest       Date:  1993-03       Impact factor: 14.808

10.  Abnormal autophagy, ubiquitination, inflammation and apoptosis are dependent upon lysosomal storage and are useful biomarkers of mucopolysaccharidosis VI.

Authors:  Alessandra Tessitore; Marinella Pirozzi; Alberto Auricchio
Journal:  Pathogenetics       Date:  2009-06-16
View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.