Disruption of bone development and homeostasis by trisomy in Ts65Dn Down syndrome mice. Bone 2011 Feb;48(2):275-80
Date
09/28/2010Pubmed ID
20870049Pubmed Central ID
PMC3021595DOI
10.1016/j.bone.2010.09.028Scopus ID
2-s2.0-78650997057 (requires institutional sign-in at Scopus site) 33 CitationsAbstract
Down syndrome (DS) is a genetic disorder resulting from trisomy 21 that causes cognitive impairment, low muscle tone and craniofacial alterations. Morphometric studies of the craniofacial and appendicular skeleton in individuals with DS suggest that bone development and homeostasis are affected by trisomy. The Ts65Dn mouse model has three copies of approximately half the genes found on human chromosome 21 and exhibits craniofacial skeletal and size differences similar to those observed in humans with DS. We hypothesized that Ts65Dn and euploid mice have distinct differences in bone development and homeostasis influencing both the craniofacial and appendicular skeletal phenotypes. Quantitative assessment of structural and mechanical properties of the femur in Ts65Dn and control mice at 6 and 16 weeks of age revealed significant deficiencies in trabecular and cortical bone architecture, bone mineral density, bone formation, and bone strength in trisomic bone. Furthermore, bone mineral density and dynamic dentin formation rate of the skull and incisor, respectively, were also reduced in Ts65Dn mice, demonstrating that trisomy significantly affects both the craniofacial and appendicular skeleton.
Author List
Blazek JD, Gaddy A, Meyer R, Roper RJ, Li JAuthor
Anna Gaddy MD Assistant Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Absorptiometry, PhotonAnimals
Bone Density
Down Syndrome
Female
Femur
Mice
Skull
Trisomy
