Osteoporosis and Osteopetrosis

Boskey, Adele L.

doi:10.1002/9783527619443.ch49

Cited by 16 publications

(15 citation statements)

References 122 publications

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“…The absence of such overprinting in our samples suggests that they were derived from a more mature animal, in which all evidence of the primary bone had been removed, except for the collagen-rich osteons associated with the vasculature. This maturation process and the bone textures it produces are distinct from those in the hypermineralized otic region in whales [28] and osteopetrosis in humans [31]. The fact that vascularization typically develops before mineralization of bone tissue suggests that the regions of present high collagen content may have been preserved from the effects of hypermineralization.…”

Section: Discussionmentioning

confidence: 99%

Hypermineralized Whale Rostrum as the Exemplar for Bone Mineral

Pasteris

Novack

2013

Connective Tissue Research

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Although bone is a nanocomposite of mineral and collagen, mineral has been the more elusive component to study. A standard for bone mineral clearly is needed. We hypothesized that the most natural, least-processed bone mineral could be retrieved from the most highly mineralized bone. We therefore studied the rostrum of the toothed whale Mesoplodon densirostris, which has the densest recognized bone. Essential to establishment of a standard for bone mineral is documentation that the proposed tissue is bone-like in all properties except for its remarkably high concentration of mineral. Transmitted-light microscopy of unstained sections of rostral material shows normal bone morphology in osteon geometry, lacunae concentration, and vasculature development. Stained sections reveal extremely low density of thin collagen fibers throughout most of the bone, but enrichment in and thicker collagen fibers around vascular holes and in a minority of osteons. FE-SEM shows the rostrum to consist mostly of dense mineral prisms. Most rostral areas have the same chemical-structural features, Raman spectroscopically dominated by strong bands at ~962 Δcm−1 and weak bands at ~2940 Δcm−1. Spectral features indicate that the rostrum is composed mainly of the calcium phosphate mineral apatite and has only about 4 wt.% organic content. The degree of carbonate substitution (~8.5 wt.% carbonate) in the apatite is in the upper range found in most types of bone. We conclude that, despite its enamel-like extraordinarily high degree of mineralization, the rostrum is in all other features bone-like. Its mineral component is the long-sought uncontaminated, unaltered exemplar of bone mineral.

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Section: Discussionmentioning

confidence: 99%

Hypermineralized Whale Rostrum as the Exemplar for Bone Mineral

Pasteris

Novack

2013

Connective Tissue Research

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“…Bone is a complex biomineralized composite material in which an organic phase-mainly composed of collagen fibers-is embedded in a calcium-phosphate matrix crystalized as hydroxyapatite (Tadic and Epple, 2004;Boskey, 2007). This complex organization was critical in conquering the earth and eventually developing the different locomotion patterns observed in terrestrial ecosystems (e.g., cursorial, braquipedial, bipedal), but also protecting the inner organs such as the nervous system (e.g., the brain is protected by the cranial vault and the spinal cord by the vertebrae) .…”

Section: Introductionmentioning

confidence: 99%

Multidisciplinary characterization of the long-bone cortex growth patterns through sheep’s ontogeny

Cambra‐Moo

Nacarino-Meneses

Dı́az-Güemes

et al. 2015

Journal of Structural Biology

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“…Similar persistence of calcified cartilage, with decreases in crystallinity and increases in mineral/matrix ratio, but without changes in collagen maturity was found in an infant with a different form of osteopetrosis [34], and decreased crystallinity was noted many years ago in x-ray diffraction analyses of a rodent model of osteopetrosis [35]. As reviewed elsewhere [36], in these cases, and in other examples of osteopetrosis not associated with cathepsin K deficiency [37,38], the growth plates similarly had increased mineral content, and calcified cartilage bars persisted in the metaphyseal bone. While it is likely that defective osteoclastic activity is the only cause of the excessive growth plate calcification, it is possible that in the absence of cathepsin K expression by hypertrophic chondrocytes [2,39], the mineralization process in the KO might be different from that in the WT.…”

Section: Discussionmentioning

confidence: 55%

Ablation of Cathepsin K Activity in the Young Mouse Causes Hypermineralization of Long Bone and Growth Plates

et al. 2009

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Cathepsin K deficiency in humans causes pycnodysostosis, which is characterized by dwarfism and osteosclerosis. Earlier studies of 10-week-old male cathepsin K-deficient (knockout, KO) mice showed their bones were mechanically more brittle, while histomorphometry showed that both osteoclasts and osteoblasts had impaired activity relative to the wildtype (WT). Here, we report detailed mineral and matrix analyses of the tibia of these animals based on Fourier Transform Infrared (FT-IR) microspectroscopy and imaging. At 10 wks, there was significant hyper-calcification of the calcified cartilage and cortices in the KO. Carbonate content was elevated in the KO calcified cartilage, cortical and cancellous bone areas These data suggest that cathepsin K does not affect mineral deposition but has a significant effect on mineralized tissue remodeling. Since growth plate abnormalities were extensive despite reported low levels of cathepsin K expression in the calcified cartilage, we used a differentiating chick-limb bud mesenchymal cell system that mimics endochondral ossification but does not contain osteoclasts to show that cathepsin K inhibition during initial stages of mineral deposition retards the mineralization process while general inhibition of cathepsins can increase mineralization. These data suggest that the hypercalcification of the cathepsin K-deficient growth plate is due to persistence of calcified cartilage and point to a role of cathepsin K in bone tissue development as well as skeletal remodeling.

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Osteoporosis and Osteopetrosis

Cited by 16 publications

References 122 publications

Hypermineralized Whale Rostrum as the Exemplar for Bone Mineral

Hypermineralized Whale Rostrum as the Exemplar for Bone Mineral

Multidisciplinary characterization of the long-bone cortex growth patterns through sheep’s ontogeny

Ablation of Cathepsin K Activity in the Young Mouse Causes Hypermineralization of Long Bone and Growth Plates

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