title: Effect of HIP/ribosomal protein L29 deficiency on mineral properties of murine bones and teeth.
abstract: Mice lacking HIP/RPL29, a component of the ribosomal machinery, display increased bone fragility. To understand the effect of sub-efficient protein synthetic rates on mineralized tissue quality, we performed dynamic and static histomorphometry and examined the mineral properties of both bones and teeth in HIP/RPL29 knock-out mice using Fourier transform infrared imaging (FTIRI). While loss of HIP/RPL29 consistently reduced total bone size, decreased mineral apposition rates were not significant, indicating that short stature is not primarily due to impaired osteoblast function. Interestingly, our microspectroscopic studies showed that a significant decrease in collagen crosslinking during maturation of HIP/RPL29-null bone precedes an overall enhancement in the relative extent of mineralization of both trabecular and cortical adult bones. This report provides strong genetic evidence that ribosomal insufficiency induces subtle organic matrix deficiencies which elevates calcification. Consistent with the HIP/RPL29-null bone phenotype, HIP/RPL29-deficient teeth also showed reduced geometric properties accompanied with relative increased mineral densities of both dentin and enamel. Increased mineralization associated with enhanced tissue fragility related to imperfection in organic phase microstructure evokes defects seen in matrix protein-related bone and tooth diseases. Thus, HIP/RPL29 mice constitute a new genetic model for studying the contribution of global protein synthesis in the establishment of organic and inorganic phases in mineral tissues.
authors.affiliation: Department of Biological Sciences, University of Delaware, Newark, DE 19716, USA.
authors.forename: Laura G
authors.initials: LG
authors.lastname: Sloofman
authors.forename: Kostas
authors.initials: K
authors.lastname: Verdelis
authors.forename: Lyudmila
authors.initials: L
authors.lastname: Spevak
authors.forename: Majd
authors.initials: M
authors.lastname: Zayzafoon
authors.forename: Mistuo
authors.initials: M
authors.lastname: Yamauchi
authors.forename: Lynn M
authors.initials: LM
authors.lastname: Opdenaker
authors.forename: Mary C
authors.initials: MC
authors.lastname: Farach-Carson
authors.forename: Adele L
authors.initials: AL
authors.lastname: Boskey
authors.forename: Catherine B
authors.initials: CB
authors.lastname: Kirn-Safran
mesh_terms: D000818:Animals; D001842:Bone and Bones / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D003094:Collagen / Q000378:metabolism; D006936:Hypercementosis / Q000000981:diagnostic imaging / Q000473:pathology; D051379:Mice; D008903:Minerals / Q000378:metabolism*; D008963:Molar / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D012269:Ribosomal Proteins / Q000172:deficiency* / Q000378:metabolism; D017550:Spectroscopy, Fourier Transform Infrared; D014070:Tooth / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D055114:X-Ray Microtomography
chemical_list: D008903:Minerals; D012269:Ribosomal Proteins; C035078:ribosomal protein L29; D003094:Collagen