Histomorphometric Analysis of the Tibial Growth Plate in a Feline Model of Mucopolysaccharidosis Type VI

1999 ◽  
Vol 65 (1) ◽  
pp. 47-52 ◽  
Author(s):  
J. D. Nuttall ◽  
L. K. Brumfield ◽  
N. L. Fazzalari ◽  
J. J. Hopwood ◽  
S. Byers
Keyword(s):  
Growth Plate ◽  
Mucopolysaccharidosis Type ◽  
Histomorphometric Analysis ◽  
Mucopolysaccharidosis Type Vi ◽  
Feline Model ◽  
Tibial Growth Plate

Effect of enzyme replacement therapy on bone formation in a feline model of mucopolysaccharidosis type VI

Bone ◽  
1997 ◽  
Vol 21 (5) ◽  
pp. 425-431 ◽  
Author(s):  
S. Byers ◽  
J.D. Nuttall ◽  
A.C. Crawley ◽  
J.J. Hopwood ◽  
K. Smith ◽  
...  
Keyword(s):  
Bone Formation ◽  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Mucopolysaccharidosis Type ◽  
Enzyme Replacement ◽  
Mucopolysaccharidosis Type Vi ◽  
Feline Model

scholarly journals Growth Plate Pathology in the Mucopolysaccharidosis Type VI Rat Model—An Experimental and Computational Approach

Diagnostics ◽  
2020 ◽  
Vol 10 (6) ◽  
pp. 360
Author(s):  
Johana M. Guevara-Morales ◽  
Michael Frohbergh ◽  
Hector Castro-Abril ◽  
Juan J. Vaca-González ◽  
Luis A. Barrera ◽  
...  
Keyword(s):  
Growth Plate ◽  
Metabolic Diseases ◽  
Histological Evaluation ◽  
Mucopolysaccharidosis Type ◽  
Mucopolysaccharidosis Type Vi ◽  
Mps Vi ◽  
In Silico Simulation ◽  
Advanced Stages ◽  
Experimental Approaches ◽  
Experimental Findings

Background: Mucopolysaccharidoses (MPS) are a group of inherited metabolic diseases caused by impaired function or absence of lysosomal enzymes involved in degradation of glycosaminoglycans. Clinically, MPS are skeletal dysplasias, characterized by cartilage abnormalities and disturbances in the process of endochondral ossification. Histologic abnormalities of growth cartilage have been reported at advanced stages of the disease, but information regarding growth plate pathology progression either in humans or in animal models, as well as its pathophysiology, is limited. Methods: Histological analyses of distal femur growth plates of wild type (WT) and mucopolysaccharidosis type VI (MPS VI) rats at different stages of development were performed, including quantitative data. Experimental findings were then analyzed in a theoretical scenario. Results: Histological evaluation showed a progressive loss of histological architecture within the growth plate. Furthermore, in silico simulation suggest the abnormal cell distribution in the tissue may lead to alterations in biochemical gradients, which may be one of the factors contributing to the growth plate abnormalities observed, highlighting aspects that must be the focus of future experimental works. Conclusion: The results presented shed some light on the progression of growth plate alterations observed in MPS VI and evidence the potentiality of combined theoretical and experimental approaches to better understand pathological scenarios, which is a necessary step to improve the search for novel therapeutic approaches.

scholarly journals Advantages of Using Same Species Enzyme for Replacement Therapy in a Feline Model of Mucopolysaccharidosis Type VI

1999 ◽  
Vol 274 (51) ◽  
pp. 36335-36343 ◽  
Author(s):  
Julie Bielicki ◽  
Allison C. Crawley ◽  
Richard C. A. Davey ◽  
Jodie C. Varnai ◽  
John J. Hopwood
Keyword(s):  
Replacement Therapy ◽  
Mucopolysaccharidosis Type ◽  
Mucopolysaccharidosis Type Vi ◽  
Feline Model

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

1997 ◽  
Vol 99 (4) ◽  
pp. 651-662 ◽  
Author(s):  
A C Crawley ◽  
K H Niedzielski ◽  
E L Isaac ◽  
R C Davey ◽  
S Byers ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Mucopolysaccharidosis Type ◽  
Enzyme Replacement ◽  
Mucopolysaccharidosis Type Vi ◽  
Feline Model

An ultrastructural study of mitotic chondrocytes in the proliferative zone of the rat tibial growth plate

1993 ◽  
Vol 175 (1) ◽  
pp. 41-45 ◽  
Author(s):  
S. Shibata ◽  
O. Baba ◽  
M. Niikura ◽  
S. Suzuki ◽  
Y. Yamashita ◽  
...  
Keyword(s):  
Growth Plate ◽  
Ultrastructural Study ◽  
Proliferative Zone ◽  
Tibial Growth Plate

Mucopolysaccharidosis Type VI (Maroteaux–Lamy syndrome) with a predominantly cardiac phenotype

2011 ◽  
Vol 104 (4) ◽  
pp. 695-699 ◽  
Author(s):  
Agnieszka Jurecka ◽  
Adam Golda ◽  
Violetta Opoka-Winiarska ◽  
Ewa Piotrowska ◽  
Anna Tylki-Szymańska
Keyword(s):  
Mucopolysaccharidosis Type ◽  
Mucopolysaccharidosis Type Vi ◽  
Cardiac Phenotype
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