Literature DB >> 6320652

Morphologic and biochemical studies of canine mucopolysaccharidosis I.

R M Shull, R G Helman, E Spellacy, G Constantopoulos, R J Munger, E F Neufeld.   

Abstract

This report presents the necropsy and biochemical findings on the first dog to die with alpha-L-iduronidase deficiency (mucopolysaccharidosis I, MPS I). Gross pathologic features, light- and electron-microscopic findings, and tissue enzyme, glycosaminoglycan (GAG), and sphingolipid levels are compared with the human disease counterpart and the previously described feline model. Results lend further support for the similarities of the canine disease and human MPS I.

Entities:  

Mesh:

Substances:

Year:  1984        PMID: 6320652      PMCID: PMC1900418     

Source DB:  PubMed          Journal:  Am J Pathol        ISSN: 0002-9440            Impact factor:   4.307


  23 in total

1.  Neurochemistry of the mucopolysaccharidoses: brain glycosaminoglycans in normals and four types of mucopolysaccharidoses.

Authors:  G Constantopoulos; R D McComb; A S Dekaban
Journal:  J Neurochem       Date:  1976-05       Impact factor: 5.372

2.  Histochemical and electron microscopic study in a case of Hurler's disease.

Authors:  D LAGUNOFF; R ROSS; E P BENDITT
Journal:  Am J Pathol       Date:  1962-09       Impact factor: 4.307

Review 3.  Ocular manifestations of the mucopolysaccharidoses.

Authors:  J François
Journal:  Ophthalmologica       Date:  1974       Impact factor: 3.250

4.  The abnormalities of lysosomal enzymes in mucopolysacc- haridoses.

Authors:  F Van Hoof; H G Hers
Journal:  Eur J Biochem       Date:  1968-12

5.  Hepatic ultrastructure in the Hurler syndrome.

Authors:  W P Callahan; A E Lorincz
Journal:  Am J Pathol       Date:  1966-02       Impact factor: 4.307

6.  Ultrastructure of cartilage in the Hurler and Sanfilippo syndromes.

Authors:  R Silberberg; D L Rimoin; R E Rosenthal; M B Hasler
Journal:  Arch Pathol       Date:  1972-12

7.  Advances in human genetics and impact on society. Conclusion.

Authors:  J F Crow
Journal:  Birth Defects Orig Artic Ser       Date:  1972-07

8.  The mucopolysaccharidoses (a review).

Authors:  A Dorfman; R Matalon
Journal:  Proc Natl Acad Sci U S A       Date:  1976-02       Impact factor: 11.205

9.  An ultrastructural comparison of normal and Hurler syndrome dermal fibroblasts.

Authors:  G W Conrad; D Sherman; A Dorfman
Journal:  Pediatr Res       Date:  1972-06       Impact factor: 3.756

10.  Mucopolysaccharidosis: secondarily induced abnormal distribution of lysosomal isoenzymes.

Authors:  J A Kint; G Dacremont; D Carton; E Orye; C Hooft
Journal:  Science       Date:  1973-07-27       Impact factor: 47.728

View more
  29 in total

1.  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

2.  Glycosaminoglycan storage in neuroanatomical regions of mucopolysaccharidosis I dogs following intrathecal recombinant human iduronidase.

Authors:  Agnes Chen; Carole Vogler; Michael McEntee; Stephen Hanson; N Matthew Ellinwood; Jackie Jens; Elizabeth Snella; Merry Passage; Steven Le; Catalina Guerra; Patricia Dickson
Journal:  APMIS       Date:  2011-05-14       Impact factor: 3.205

3.  Neonatal Systemic AAV Induces Tolerance to CNS Gene Therapy in MPS I Dogs and Nonhuman Primates.

Authors:  Christian Hinderer; Peter Bell; Jean-Pierre Louboutin; Yanqing Zhu; Hongwei Yu; Gloria Lin; Ruth Choa; Brittney L Gurda; Jessica Bagel; Patricia O'Donnell; Tracey Sikora; Therese Ruane; Ping Wang; Alice F Tarantal; Margret L Casal; Mark E Haskins; James M Wilson
Journal:  Mol Ther       Date:  2015-05-29       Impact factor: 11.454

4.  A model of mucopolysaccharidosis IIIB (Sanfilippo syndrome type IIIB): N-acetyl-alpha-D-glucosaminidase deficiency in Schipperke dogs.

Authors:  N M Ellinwood; P Wang; T Skeen; N J H Sharp; M Cesta; S Decker; N J Edwards; I Bublot; J N Thompson; W Bush; E Hardam; M E Haskins; U Giger
Journal:  J Inherit Metab Dis       Date:  2003       Impact factor: 4.982

5.  Enzyme replacement in a canine model of Hurler syndrome.

Authors:  R M Shull; E D Kakkis; M F McEntee; S A Kania; A J Jonas; E F Neufeld
Journal:  Proc Natl Acad Sci U S A       Date:  1994-12-20       Impact factor: 11.205

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

Authors:  Mark Haskins
Journal:  ILAR J       Date:  2009

Review 7.  Animal models for metabolic, neuromuscular and ophthalmological rare diseases.

Authors:  Guillaume Vaquer; Frida Rivière; Maria Mavris; Fabrizia Bignami; Jordi Llinares-Garcia; Kerstin Westermark; Bruno Sepodes
Journal:  Nat Rev Drug Discov       Date:  2013-03-15       Impact factor: 84.694

8.  Characterization of an immunodeficient mouse model of mucopolysaccharidosis type I suitable for preclinical testing of human stem cell and gene therapy.

Authors:  Mayra F Garcia-Rivera; Leah E Colvin-Wanshura; Matthew S Nelson; Zhenhong Nan; Shaukat A Khan; Tyson B Rogers; Indrani Maitra; Walter C Low; Pankaj Gupta
Journal:  Brain Res Bull       Date:  2007-08-06       Impact factor: 4.077

9.  Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

Authors:  Dan Wang; Charu Shukla; Xiaoli Liu; Trenton R Schoeb; Lorne A Clarke; David M Bedwell; Kim M Keeling
Journal:  Mol Genet Metab       Date:  2010-01       Impact factor: 4.797

Review 10.  Animal models for mucopolysaccharidosis disorders and their clinical relevance.

Authors:  Mark E Haskins
Journal:  Acta Paediatr       Date:  2007-04       Impact factor: 2.299

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.