title: Connective tissue mineralization in Abcc6-/- mice, a model for pseudoxanthoma elasticum.
abstract: Pseudoxanthoma elasticum (PXE) is a heritable multisystem disorder characterized by ectopic mineralization. However, the structure of the mineral deposits, their interactions with the connective tissue matrix, and the details of the progressive maturation of the mineral crystals are currently unknown. In this study, we examined the mineralization processes in Abcc6(-/-) mice, a model system for PXE, by energy dispersive X-ray and Fourier transform infrared imaging spectroscopy (FT-IRIS). The results indicated that the principal components of the mineral deposits were calcium and phosphate which co-localized within the histologically demonstrable lesions determined by topographic mapping. The Ca/P ratio increased in samples with progressive mineralization reaching the value comparable to that in endochondral bone. A progressive increase in mineralization was also reflected by increased mineral-to-matrix ratio determined by FT-IRIS. Determination of the mineral phases by FT-IRIS suggested progressive maturation of the mineral deposits from amorphous calcium phosphate to hydroxyapatite. These results provide critical information of the mechanisms of mineralization in PXE, with potential pharmacologic implications.
authors.affiliation: Department of Bioengineering, Temple University, Philadelphia, PA 19122, USA.
authors.forename: N Beril
authors.initials: NB
authors.lastname: Kavukcuoglu
authors.forename: Qiaoli
authors.initials: Q
authors.lastname: Li
authors.forename: Nancy
authors.initials: N
authors.lastname: Pleshko
authors.forename: Jouni
authors.initials: J
authors.lastname: Uitto
mesh_terms: D000818:Animals; D002114:Calcinosis / Q000473:pathology*; D002118:Calcium / Q000378:metabolism; D002130:Calcium Phosphates / Q000378:metabolism; D003238:Connective Tissue / Q000378:metabolism / Q000473:pathology*; D017886:Durapatite / Q000378:metabolism; D006321:Heart / Q000294:innervation; D007668:Kidney / Q000294:innervation / Q000473:pathology; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009999:Ossification, Heterotopic; D010710:Phosphates / Q000378:metabolism; D011561:Pseudoxanthoma Elasticum / Q000378:metabolism / Q000473:pathology*; D017550:Spectroscopy, Fourier Transform Infrared; D013997:Time Factors; D014738:Vibrissae / Q000378:metabolism / Q000473:pathology
chemical_list: D002130:Calcium Phosphates; D010710:Phosphates; C519480:amorphous calcium phosphate; D017886:Durapatite; D002118:Calcium