Analysis Tools
growth/size/body
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• incisors show a thin cementum layer, PDL fiber disorganization, and PDL detachment from the root surface
• at P26, incisors display epithelial in-growth to the periodontal region of the lingual root analog, in association with PDL detachment
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• mice display a high incidence of incisor malocclusion
(J:234764)
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• molars exhibit a lack of functional acellular cementum formation and subsequent loss of Sharpey's collagen fiber insertion into the tooth root structure
• however, molar dentin formation is normal, with no delay in mineralization, no alteration in dentin dimensions, and no differences in odontoblast markers during crown (P1) or root (P14) dentinogenesis
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• at 10 weeks of age, molars show abundant pitting concentrated on the cervical portion of the root surface
• molar roots display exposed dentinal tubules, indicating the absence of cementum in regions of root resorption
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• mice show a significant reduction in acellular cementum formation on molar and incisor roots
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• mice display a high incidence of incisor malocclusion
(J:234764)
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• mice show progressive breakdown of periodontal organization, with increased incidence of root resorption and increased numbers of osteoclast-like cells, RANKL immunostaining, and bone resorption in alveolar bone by P60
• however, there is no neutrophil infiltration into the periodontia
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• areas of bone loss are apparent in alveolar bone at P26
(J:228156)
• severe alveolar bone resorption by P60
(J:228156)
• mice show increased bone resorption in alveolar bone by P60
(J:234764)
• mandibles show extensive reduction in alveolar bone height, particularly along the lingual aspect
(J:234764)
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• at P14 and P27, molars show prevalent periodontal ligament (PDL) detachment from the root surface; in regions where the ligament remains attached, PDL organization appears relatively normal
(J:228156)
• SEM and TEM analyses revealed the presence of poorly embedded Sharpey's fibers into the tooth root structure, with detachment and disorganization of collagen fibrils
(J:228156)
• however, at the PDL-bone interface, Sharpey's fibers insertion appears normal and no detachment is observed
(J:228156)
• at P26, molars show PDL disorganization in the cervical root and defects in the cementum-PDL interface as shown by progressive loss of PDL attachment from the root surface; by P60, PDL detachment is seen in all molars examined
(J:234764)
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• at P26, molars show delayed mineralization of the apical cellular cementum relative to wild-type controls; however, the amount of cellular cementum covering the apical molar root is normal by P60
• at P60, molars show no mineralized cementum ultrastructure on the cervical root surfaces, indicating failure of acellular cementum mineralization
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• at P14 and P27-30, molars show no layer of acellular cementum adjacent to the mantle dentin layer, resulting in a disrupted dentin-PDL interface
(J:228156)
• mice show a significant reduction in acellular cementum formation on molar and incisor roots
(J:234764)
• at P14, molars show a significantly thinner layer of acellular cementum covering the root dentin; both buccal and lingual acellular cementum shows stunted growth at P26 and P60, unlike in wild-type molars
(J:234764)
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• at P26, molars show abnormal thickening of the junctional epithelium in association PDL detachment
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• the size of both male and female mutant mice is lower than controls at 4 months and up to 16 months of age
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• the weight of both male and female mutant mice is lower than controls at 4 months and up to 16 months of age
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skeleton
| N |
• no difference in growth plate thickness in adult (46 mo old) mutant and control mice was detectable
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• at P5, the coronal suture is significantly larger by 65% relative to that in wild-type controls; however, no differences are noted at P14, and by P26 coronal suture width is significantly reduced
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• at P5, the lambdoid suture is significantly larger by 20% relative to that in wild-type controls; however, no differences are noted at P14, and by P26 lambdoid suture width is significantly reduced
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• at P5, the anterior fontanelle width is significantly increased by 40%; however, no differences are noted at P14, and by P26 the anterior fontanelle width trends towards reduction
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• areas of bone loss are apparent in alveolar bone at P26
(J:228156)
• severe alveolar bone resorption by P60
(J:228156)
• mice show increased bone resorption in alveolar bone by P60
(J:234764)
• mandibles show extensive reduction in alveolar bone height, particularly along the lingual aspect
(J:234764)
|
|
• incisors show a thin cementum layer, PDL fiber disorganization, and PDL detachment from the root surface
• at P26, incisors display epithelial in-growth to the periodontal region of the lingual root analog, in association with PDL detachment
|
|
• mice display a high incidence of incisor malocclusion
(J:234764)
|
|
• molars exhibit a lack of functional acellular cementum formation and subsequent loss of Sharpey's collagen fiber insertion into the tooth root structure
• however, molar dentin formation is normal, with no delay in mineralization, no alteration in dentin dimensions, and no differences in odontoblast markers during crown (P1) or root (P14) dentinogenesis
|
|
• at 10 weeks of age, molars show abundant pitting concentrated on the cervical portion of the root surface
• molar roots display exposed dentinal tubules, indicating the absence of cementum in regions of root resorption
|
|
• at P14 and P27, molars show prevalent periodontal ligament (PDL) detachment from the root surface; in regions where the ligament remains attached, PDL organization appears relatively normal
(J:228156)
• SEM and TEM analyses revealed the presence of poorly embedded Sharpey's fibers into the tooth root structure, with detachment and disorganization of collagen fibrils
(J:228156)
• however, at the PDL-bone interface, Sharpey's fibers insertion appears normal and no detachment is observed
(J:228156)
• at P26, molars show PDL disorganization in the cervical root and defects in the cementum-PDL interface as shown by progressive loss of PDL attachment from the root surface; by P60, PDL detachment is seen in all molars examined
(J:234764)
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• at P26, molars show delayed mineralization of the apical cellular cementum relative to wild-type controls; however, the amount of cellular cementum covering the apical molar root is normal by P60
• at P60, molars show no mineralized cementum ultrastructure on the cervical root surfaces, indicating failure of acellular cementum mineralization
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• mice show a significant reduction in acellular cementum formation on molar and incisor roots
|
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• at P14 and P27-30, molars show no layer of acellular cementum adjacent to the mantle dentin layer, resulting in a disrupted dentin-PDL interface
(J:228156)
• mice show a significant reduction in acellular cementum formation on molar and incisor roots
(J:234764)
• at P14, molars show a significantly thinner layer of acellular cementum covering the root dentin; both buccal and lingual acellular cementum shows stunted growth at P26 and P60, unlike in wild-type molars
(J:234764)
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• mice display a high incidence of incisor malocclusion
(J:234764)
|
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• mice show severe mandibular bone resorption by P60
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• osteoclast surfaces are reduced in mutant mice compared with control femurs of both sexes
(J:136223)
• expression levels of osteoclast markers Acp5 and Tnfrsf11a are significantly decreased in calvarial bone tissues at P14, with similar trends at P26, suggesting that reduced osteoclast activity may contribute to decreased cranial suture widths
(J:228156)
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• in tibia, osteoclast numbers are found to be significantly reduced in mutant mice
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• although alveolar and mandibular bones exhibit equivalent or fewer osteoclasts at early ages (P1 and P14), by P26 alveolar bone shows a 2-fold increase in the number of osteoclasts relative to wild-type tissues
• by P60, the number of osteoclasts in alveolar and alveolar/mandibular bone is increased by 8-fold and 3-fold, respectively
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• thinner cortices in femurs of 4-month-old mutant mice
• with age cortical thickness of mutant bones reaches a size equivalent to that of controls
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short femur
(
J:136223
)
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• at 4 months
• remain short over time
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• bone mineral density (BMD) of femurs of mutant mice is 20% lower than in control mice at 4 months of age, but equivalent in 12 month old mice
• midshaft femoral cortical bone showed that bone matrix mineralization is 9% lower in mutant mice compared with controls at birth (embryonic day E18 fetuses), and 5% lower at 4 months of age, but not significantly different at 12 months of age
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• expression levels of osteoblast markers Alpl/TNAP and Spp1/OPN are significantly decreased in calvarial bone tissues at P14, with similar trends at P26
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• reduced surfaces of cuboidal (plump) osteoblasts
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• trabecular bone volume (BV/TV) in long bones of mutant male and female mice is 2540% higher than controls
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• higher trabecular number (TbN) in mutant versus control bones
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• lower trabecular separation in mutant versus control bones
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• trabecular thickness tends to be smaller in mutant mice
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• osteoid surface is increased
(J:136223)
• at P1, alveolar and mandibular bones show extensive regions of unmineralized osteoid accumulation
(J:228156)
• at P14, extensive osteoid accumulation is detected on the surfaces of bone of the alveolar crest and molar root furcation region
(J:228156)
• osteoid is still detectable on alveolar bone surfaces at P26 and P60
(J:228156)
• however, neither frank osteomalacia nor accumulation of osteoid on cortical or trabecular bone surfaces are identified in femurs at P30
(J:228156)
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• delayed primary mineralization
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• at P1, mice show delayed intramembranous bone ossification in the alveolus surrounding the molar tooth bud, the basal bone of the mandible, and the coronal suture region relative to wild-type controls
• delay in intramembranous mineralization persists at P14, as shown by extensive unmineralized osteoid on the surfaces of bone of the alveolar crest and molar root furcation region
• however, no major defects are identified in endochondral ossification in the cranial base, and synchondroses appear normal in structure and matrix content
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• mice exhibit wider cranial sutures than wild-type controls at P5, no apparent differences at P14, and more closed sutures (but not fusion) by P26, indicating that the early mineralization defect reflected in suture widths is normalized and then reversed at later ages
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• mice exhibit wider fontanelles than wild-type controls at P5, no apparent differences at P14, and more closed fontanelles by P26
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• very low bone formation activity in 4-month-old male and female mutant mice compared with controls
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• mice show severe alveolar and mandibular bone resorption by P60
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• at P30, molars show resorption pits on the root surface
(J:228156)
• at 10 weeks of age, molars exhibit extensive pitting on the cervical portion of the root surface, consistent with external root resorption
(J:234764)
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hematopoietic system
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• osteoclast surfaces are reduced in mutant mice compared with control femurs of both sexes
(J:136223)
• expression levels of osteoclast markers Acp5 and Tnfrsf11a are significantly decreased in calvarial bone tissues at P14, with similar trends at P26, suggesting that reduced osteoclast activity may contribute to decreased cranial suture widths
(J:228156)
|
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• in tibia, osteoclast numbers are found to be significantly reduced in mutant mice
|
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• although alveolar and mandibular bones exhibit equivalent or fewer osteoclasts at early ages (P1 and P14), by P26 alveolar bone shows a 2-fold increase in the number of osteoclasts relative to wild-type tissues
• by P60, the number of osteoclasts in alveolar and alveolar/mandibular bone is increased by 8-fold and 3-fold, respectively
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craniofacial
| N |
• mice exhibit no significant changes in craniofacial size and shape, supporting histological evidence that the cranial base forms and mineralizes normally
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• at P5, the coronal suture is significantly larger by 65% relative to that in wild-type controls; however, no differences are noted at P14, and by P26 coronal suture width is significantly reduced
|
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• at P5, the lambdoid suture is significantly larger by 20% relative to that in wild-type controls; however, no differences are noted at P14, and by P26 lambdoid suture width is significantly reduced
|
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• at P5, the anterior fontanelle width is significantly increased by 40%; however, no differences are noted at P14, and by P26 the anterior fontanelle width trends towards reduction
|
|
• incisors show a thin cementum layer, PDL fiber disorganization, and PDL detachment from the root surface
• at P26, incisors display epithelial in-growth to the periodontal region of the lingual root analog, in association with PDL detachment
|
|
• mice display a high incidence of incisor malocclusion
(J:234764)
|
|
• molars exhibit a lack of functional acellular cementum formation and subsequent loss of Sharpey's collagen fiber insertion into the tooth root structure
• however, molar dentin formation is normal, with no delay in mineralization, no alteration in dentin dimensions, and no differences in odontoblast markers during crown (P1) or root (P14) dentinogenesis
|
|
• at 10 weeks of age, molars show abundant pitting concentrated on the cervical portion of the root surface
• molar roots display exposed dentinal tubules, indicating the absence of cementum in regions of root resorption
|
|
• mice show a significant reduction in acellular cementum formation on molar and incisor roots
|
|
• mice display a high incidence of incisor malocclusion
(J:234764)
|
|
• mice show severe mandibular bone resorption by P60
|
|
• mice show progressive breakdown of periodontal organization, with increased incidence of root resorption and increased numbers of osteoclast-like cells, RANKL immunostaining, and bone resorption in alveolar bone by P60
• however, there is no neutrophil infiltration into the periodontia
|
|
• areas of bone loss are apparent in alveolar bone at P26
(J:228156)
• severe alveolar bone resorption by P60
(J:228156)
• mice show increased bone resorption in alveolar bone by P60
(J:234764)
• mandibles show extensive reduction in alveolar bone height, particularly along the lingual aspect
(J:234764)
|
|
• at P14 and P27, molars show prevalent periodontal ligament (PDL) detachment from the root surface; in regions where the ligament remains attached, PDL organization appears relatively normal
(J:228156)
• SEM and TEM analyses revealed the presence of poorly embedded Sharpey's fibers into the tooth root structure, with detachment and disorganization of collagen fibrils
(J:228156)
• however, at the PDL-bone interface, Sharpey's fibers insertion appears normal and no detachment is observed
(J:228156)
• at P26, molars show PDL disorganization in the cervical root and defects in the cementum-PDL interface as shown by progressive loss of PDL attachment from the root surface; by P60, PDL detachment is seen in all molars examined
(J:234764)
|
|
• at P26, molars show delayed mineralization of the apical cellular cementum relative to wild-type controls; however, the amount of cellular cementum covering the apical molar root is normal by P60
• at P60, molars show no mineralized cementum ultrastructure on the cervical root surfaces, indicating failure of acellular cementum mineralization
|
|
• at P14 and P27-30, molars show no layer of acellular cementum adjacent to the mantle dentin layer, resulting in a disrupted dentin-PDL interface
(J:228156)
• mice show a significant reduction in acellular cementum formation on molar and incisor roots
(J:234764)
• at P14, molars show a significantly thinner layer of acellular cementum covering the root dentin; both buccal and lingual acellular cementum shows stunted growth at P26 and P60, unlike in wild-type molars
(J:234764)
|
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• at P26, molars show abnormal thickening of the junctional epithelium in association PDL detachment
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digestive/alimentary system
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• at P26, molars show abnormal thickening of the junctional epithelium in association PDL detachment
|
immune system
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• osteoclast surfaces are reduced in mutant mice compared with control femurs of both sexes
(J:136223)
• expression levels of osteoclast markers Acp5 and Tnfrsf11a are significantly decreased in calvarial bone tissues at P14, with similar trends at P26, suggesting that reduced osteoclast activity may contribute to decreased cranial suture widths
(J:228156)
|
|
• in tibia, osteoclast numbers are found to be significantly reduced in mutant mice
|
|
• although alveolar and mandibular bones exhibit equivalent or fewer osteoclasts at early ages (P1 and P14), by P26 alveolar bone shows a 2-fold increase in the number of osteoclasts relative to wild-type tissues
• by P60, the number of osteoclasts in alveolar and alveolar/mandibular bone is increased by 8-fold and 3-fold, respectively
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limbs/digits/tail
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• thinner cortices in femurs of 4-month-old mutant mice
• with age cortical thickness of mutant bones reaches a size equivalent to that of controls
|
short femur
(
J:136223
)
|
• at 4 months
• remain short over time
|
