Biomaterial Database

Sclerostin in mineralized matrices and van Buchem disease. 2009

R L van Bezooijen, and A L Bronckers, and R A Gortzak, and P C W Hogendoorn, and L van der Wee-Pals, and W Balemans, and H J Oostenbroek, and W Van Hul, and H Hamersma, and F G Dikkers, and N A T Hamdy, and S E Papapoulos, and C W G M Löwik

Departments of Endocrinology and Metabolic Diseases, C4R, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands. R.L.van_Bezooyen@lumc.nl

Sclerostin is an inhibitor of bone formation expressed by osteocytes. We hypothesized that sclerostin is expressed by cells of the same origin and also embedded within mineralized matrices. In this study, we analyzed (a) sclerostin expression using immunohistochemistry, (b) whether the genomic defect in individuals with van Buchem disease (VBD) was associated with the absence of sclerostin expression, and (c) whether this was associated with hypercementosis. Sclerostin was expressed by cementocytes in mouse and human teeth and by mineralized hypertrophic chondrocytes in the human growth plate. In individuals with VBD, sclerostin expression was absent or strongly decreased in osteocytes and cementocytes. This was associated with increased bone formation, but no overt changes in cementum thickness. In conclusion, sclerostin is expressed by all 3 terminally differentiated cell types embedded within mineralized matrices: osteocytes, cementocytes, and hypertrophic chondrocytes.

UI	MeSH Term	Description	Entries
D007569	Jaw Abnormalities	Congenital absence of or defects in structures of the jaw.	Abnormalities, Jaw,Abnormality, Jaw,Jaw Abnormality
D008297	Male		Males
D008310	Malocclusion	Such malposition and contact of the maxillary and mandibular teeth as to interfere with the highest efficiency during the excursive movements of the jaw that are essential for mastication. (Jablonski, Illustrated Dictionary of Dentistry, 1982)	Angle's Classification,Crossbite,Tooth Crowding,Cross Bite,Angle Classification,Angles Classification,Bite, Cross,Bites, Cross,Classification, Angle's,Cross Bites,Crossbites,Crowding, Tooth,Crowdings, Tooth,Malocclusions
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D010011	Osteocytes	Mature osteoblasts that have become embedded in the BONE MATRIX. They occupy a small cavity, called lacuna, in the matrix and are connected to adjacent osteocytes via protoplasmic projections called canaliculi.	Osteocyte
D010026	Osteosclerosis	An abnormal hardening or increased density of bone tissue.	Osteoscleroses
D011862	Radiography, Panoramic	Extraoral body-section radiography depicting an entire maxilla, or both maxilla and mandible, on a single film.	Orthopantomography,Panoramic Radiography,Pantomography,Orthopantomographies,Panoramic Radiographies,Pantomographies,Radiographies, Panoramic
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003739	Dental Cementum	The bonelike rigid connective tissue covering the root of a tooth from the cementoenamel junction to the apex and lining the apex of the root canal, also assisting in tooth support by serving as attachment structures for the periodontal ligament. (Jablonski, Dictionary of Dentistry, 1992)	Cementoblasts,Cementum,Cementoblast,Cementum, Dental
D005260	Female		Females