scholarly journals Abnormal bone quality in cartilage oligomeric matrix protein and matrilin 3 double-deficient mice caused by increased tissue inhibitor of metalloproteinases 3 deposition and delayed aggrecan degradation

2012 ◽  
Vol 64 (8) ◽  
pp. 2644-2654 ◽  
Author(s):  
Gergely Groma ◽  
Wei Xin ◽  
Ivan Grskovic ◽  
Anja Niehoff ◽  
Bent Brachvogel ◽  
...  
2002 ◽  
Vol 22 (12) ◽  
pp. 4366-4371 ◽  
Author(s):  
Liz Svensson ◽  
Attila Aszódi ◽  
Dick Heinegård ◽  
Ernst B. Hunziker ◽  
Finn P. Reinholt ◽  
...  

ABSTRACT Cartilage oligomeric matrix protein (COMP) belongs to the thrombospondin family and is a homopentamer primarily expressed in cartilage. Mutations in the COMP gene result in the autosomal dominant chondrodysplasias pseudoachondroplasia (PSACH) and some types of multiple epiphyseal dysplasia (MED), which are characterized by mild to severe short-limb dwarfism and early-onset osteoarthritis. We have generated COMP-null mice to study the role of COMP in vivo. These mice show no anatomical, histological, or ultrastructural abnormalities and show none of the clinical signs of PSACH or MED. Northern blot analysis and immunohistochemical analysis of cartilage indicate that the lack of COMP is not compensated for by any other member of the thrombospondin family. The results also show that the phenotype in PSACH/MED cartilage disorders is not caused by the reduced amount of COMP.


2015 ◽  
Vol 11 (999) ◽  
pp. 1-1
Author(s):  
Maurizio Benucci ◽  
Francesca Meacci ◽  
Mariangela Manfredi ◽  
Francesca Gobbi ◽  
Maria Infantino ◽  
...  

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