Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmp1tm1Mis mutation
(0 available);
any
Dmp1 mutation
(42 available)
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skeleton
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• maturation of woven bone is defective
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• secondary ossification is delayed
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limbs/digits/tail
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmp1tm1Mis mutation
(0 available);
any
Dmp1 mutation
(42 available)
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Abnormal tooth morphology and mineralization in Dmp1tm1Mis/Dmp1tm1Mis pups
craniofacial
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• upper first molars show enlarged pulp chambers and root canals by 1 month of age
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• the third molar is either absent or retarded in about 10% of mice
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• teeth show reduced mineral content after 3 days of age
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• cementum is malformed and shows defective mineralization with immature calcospherites
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• delayed development of the third molar in about 10% of mice
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• dentin layer is reduced at 3 days of age
(J:89498)
• upper first molars show reduced dentin root thickness by 1 month of age and a dramatic change of dentin matrix structure at 3 months of age
(J:89498)
• upper first molars show a poorly organized dentin peritubular ultrastructure with increased spaces and a coarse and irregular dentin surface at 3 months of age
(J:89498)
• dentin zone is decreased in 1- and 12-month upper first molars and 3-month incisors
(J:89498)
• signs of dentin defects (enlarged pulp and thin dentin) are obvious at 3 weeks of age
(J:241769)
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• both incisors and molars show partial failure of maturation of predentin into dentin during postnatal development
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• upper first molars show a progressive reduction in root dentin thickness and mineral density after 3 weeks, with a severe decrease in mineralization at 3 months of age
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• predentin layer is reduced at 3 days of age
• width of predentin zone is expanded in 1- and 12-month upper first molars and 3-month incisors
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• enamel layer is reduced at 3 days of age
• enamel layer is thinner in 1- and 12-month upper first molars and 3-month incisors
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• misalignment of teeth due to severe alveolar bone loss
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• mice develop an early-onset periodontal breakdown, with a severe alveolar bone defect and periodontal ligament (PDL)/cementum detachment at 3 and 6 months of age
• periodontal breakdown worsens with age, esp. in the interproximal area between molars
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• mineral content of alveolar bone appears to be increased at 3 days of age
(J:89498)
• alveolar bone appears woven and immature at 3 weeks and 3 months of age, with more osteoblasts and large bone marrow spaces, suggesting impaired maturation of woven bone into lamellar bone
(J:241769)
• porous, hypomineralized alveolar bone is evident at 3 and 6 months of age, with extreme porosity seen by 12 months
(J:241769)
• dramatic increase in proteoglycans (biglycan and decorin) and defective osteocyte lacuno-canalicular system in alveolar bone at 3 weeks and 3 months of age
(J:241769)
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• significant decrease of alveolar bone width and alveolar bone area between the first and second molars at 3 months, but not at 3 weeks of age; however, alveolar bone height is normal, suggesting the defect in interproximal bone is mainly vertical
• severe alveolar bone loss by 12 months of age
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• detachment between the PDL and cementum at 3 months of age
• the PDL layer is composed of fewer and mostly spindle-shaped fibroblasts, unlike in wild-type controls where PDL cells are mixed with few spindle-like shapes
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• cementoblasts are fewer in number and no longer cuboid, but spindle-shaped
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• cementoblasts are fewer in number
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• the acellular cementum layer is much thinner and more difficult to discern
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• increased expression of biglycan and decorin in cellular cementum at 3 weeks and 3 months of age
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• fewer cementocyte dendrites
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• cementocyte canaliculi are reduced in number
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• the cementocyte lacuna is rough on the lacunar wall, with few dendrites
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immune system
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• mice are more prone to bacterial infection upon aging (1 yr); bacterial colonies, infiltrated lymphocytes and neutrophils, and detritus such as hair shafts are present in 12-month-old mandibles, most likely due to alveolar bone loss and PDL detachment
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skeleton
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• the overall development of the skeleton in mouse fetuses and newborns was grossly normal
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• mineral content of alveolar bone appears to be increased at 3 days of age
(J:89498)
• alveolar bone appears woven and immature at 3 weeks and 3 months of age, with more osteoblasts and large bone marrow spaces, suggesting impaired maturation of woven bone into lamellar bone
(J:241769)
• porous, hypomineralized alveolar bone is evident at 3 and 6 months of age, with extreme porosity seen by 12 months
(J:241769)
• dramatic increase in proteoglycans (biglycan and decorin) and defective osteocyte lacuno-canalicular system in alveolar bone at 3 weeks and 3 months of age
(J:241769)
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• significant decrease of alveolar bone width and alveolar bone area between the first and second molars at 3 months, but not at 3 weeks of age; however, alveolar bone height is normal, suggesting the defect in interproximal bone is mainly vertical
• severe alveolar bone loss by 12 months of age
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• upper first molars show enlarged pulp chambers and root canals by 1 month of age
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• the third molar is either absent or retarded in about 10% of mice
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• detachment between the PDL and cementum at 3 months of age
• the PDL layer is composed of fewer and mostly spindle-shaped fibroblasts, unlike in wild-type controls where PDL cells are mixed with few spindle-like shapes
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• cementoblasts are fewer in number and no longer cuboid, but spindle-shaped
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• cementoblasts are fewer in number
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• teeth show reduced mineral content after 3 days of age
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• cementum is malformed and shows defective mineralization with immature calcospherites
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• delayed development of the third molar in about 10% of mice
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• the acellular cementum layer is much thinner and more difficult to discern
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• increased expression of biglycan and decorin in cellular cementum at 3 weeks and 3 months of age
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• fewer cementocyte dendrites
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• cementocyte canaliculi are reduced in number
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• the cementocyte lacuna is rough on the lacunar wall, with few dendrites
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• dentin layer is reduced at 3 days of age
(J:89498)
• upper first molars show reduced dentin root thickness by 1 month of age and a dramatic change of dentin matrix structure at 3 months of age
(J:89498)
• upper first molars show a poorly organized dentin peritubular ultrastructure with increased spaces and a coarse and irregular dentin surface at 3 months of age
(J:89498)
• dentin zone is decreased in 1- and 12-month upper first molars and 3-month incisors
(J:89498)
• signs of dentin defects (enlarged pulp and thin dentin) are obvious at 3 weeks of age
(J:241769)
|
|
|
• both incisors and molars show partial failure of maturation of predentin into dentin during postnatal development
|
|
|
• upper first molars show a progressive reduction in root dentin thickness and mineral density after 3 weeks, with a severe decrease in mineralization at 3 months of age
|
|
|
• predentin layer is reduced at 3 days of age
• width of predentin zone is expanded in 1- and 12-month upper first molars and 3-month incisors
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• enamel layer is reduced at 3 days of age
• enamel layer is thinner in 1- and 12-month upper first molars and 3-month incisors
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• misalignment of teeth due to severe alveolar bone loss
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• modest expansion of the hypertrophic chondrocyte zone in fetuses at E18
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• modest increase in long bone diameter
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• increased numbers of osteoblasts in alveolar bone at 3 weeks and 3 months of age
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• disorganized osteocyte lacuno-canalicular system in alveolar bone at 3 weeks and 3 months of age
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• fewer osteocyte canaliculi with rough canalicular walls in alveolar bone; canaliculi are less straight and smooth
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• osteocyte lacunae have rough walls and are larger in size in alveolar bone; distribution of the lacunae appears less organized and more clustered
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• increased amount of osteoid present in the matrix in alveolar bone at 3 weeks and 3 months of age
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• alveolar bone is woven and immature at 3 weeks and 3 months of age, with more osteoblasts and large bone marrow spaces, suggesting impaired maturation of woven bone into lamellar bone
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• porous, hypomineralized alveolar bone is evident at 3 and 6 months of age, with extreme porosity seen by 12 months
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growth/size/body
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• upper first molars show enlarged pulp chambers and root canals by 1 month of age
|
|
|
• the third molar is either absent or retarded in about 10% of mice
|
|
|
• teeth show reduced mineral content after 3 days of age
|
|
|
• cementum is malformed and shows defective mineralization with immature calcospherites
|
|
|
• delayed development of the third molar in about 10% of mice
|
|
|
• dentin layer is reduced at 3 days of age
(J:89498)
• upper first molars show reduced dentin root thickness by 1 month of age and a dramatic change of dentin matrix structure at 3 months of age
(J:89498)
• upper first molars show a poorly organized dentin peritubular ultrastructure with increased spaces and a coarse and irregular dentin surface at 3 months of age
(J:89498)
• dentin zone is decreased in 1- and 12-month upper first molars and 3-month incisors
(J:89498)
• signs of dentin defects (enlarged pulp and thin dentin) are obvious at 3 weeks of age
(J:241769)
|
|
|
• both incisors and molars show partial failure of maturation of predentin into dentin during postnatal development
|
|
|
• upper first molars show a progressive reduction in root dentin thickness and mineral density after 3 weeks, with a severe decrease in mineralization at 3 months of age
|
|
|
• predentin layer is reduced at 3 days of age
• width of predentin zone is expanded in 1- and 12-month upper first molars and 3-month incisors
|
|
|
• enamel layer is reduced at 3 days of age
• enamel layer is thinner in 1- and 12-month upper first molars and 3-month incisors
|
|
|
• misalignment of teeth due to severe alveolar bone loss
|
|
|
• mice develop an early-onset periodontal breakdown, with a severe alveolar bone defect and periodontal ligament (PDL)/cementum detachment at 3 and 6 months of age
• periodontal breakdown worsens with age, esp. in the interproximal area between molars
|
|
|
• mineral content of alveolar bone appears to be increased at 3 days of age
(J:89498)
• alveolar bone appears woven and immature at 3 weeks and 3 months of age, with more osteoblasts and large bone marrow spaces, suggesting impaired maturation of woven bone into lamellar bone
(J:241769)
• porous, hypomineralized alveolar bone is evident at 3 and 6 months of age, with extreme porosity seen by 12 months
(J:241769)
• dramatic increase in proteoglycans (biglycan and decorin) and defective osteocyte lacuno-canalicular system in alveolar bone at 3 weeks and 3 months of age
(J:241769)
|
|
|
• significant decrease of alveolar bone width and alveolar bone area between the first and second molars at 3 months, but not at 3 weeks of age; however, alveolar bone height is normal, suggesting the defect in interproximal bone is mainly vertical
• severe alveolar bone loss by 12 months of age
|
|
|
• detachment between the PDL and cementum at 3 months of age
• the PDL layer is composed of fewer and mostly spindle-shaped fibroblasts, unlike in wild-type controls where PDL cells are mixed with few spindle-like shapes
|
|
|
• cementoblasts are fewer in number and no longer cuboid, but spindle-shaped
|
|
|
• cementoblasts are fewer in number
|
|
|
• the acellular cementum layer is much thinner and more difficult to discern
|
|
|
• increased expression of biglycan and decorin in cellular cementum at 3 weeks and 3 months of age
|
|
|
• fewer cementocyte dendrites
|
|
|
• cementocyte canaliculi are reduced in number
|
|
|
• the cementocyte lacuna is rough on the lacunar wall, with few dendrites
|
|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmp1tm1Mis mutation
(0 available);
any
Dmp1 mutation
(42 available)
|
|
|
cardiovascular system
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• delay in blood vessel formation in bones at E11
• mice injected with anti-Fgf23 antibodies show rescue of the delay
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homeostasis/metabolism
cellular
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• osteoblasts continuously express osteoblast-specific genes and fail to differentiate into mature osteocytes
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renal/urinary system
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• increase in renal phosphorous clearance
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skeleton
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• the number of osteoclasts formed in bone marrow cultures in the presence of 1,25-(OH)2D3 is less than 50% of that in control
• marker analysis indicates a reduction in osteoclasts in long bone in both the epiphysis and metaphysis at 2 weeks of age
• however, the number of osteoclasts formed by spleen cells is normal, indicating that osteoclast precursors can differentiate normally
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• expansion of the metaphysis
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• abnormalities in cortical bone
• mice treated with anti-Fgf13 antibodies show improvement in cortical bone abnormalities
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• defective organization of osteocyte lacunae and lacunocanalicular walls
• the inner lacunocanalicular wall is buckled and enlarged rather than smooth in the poorly mineralized matrix, unmineralized collagen fibrils are seen, the lamina limitans is absent, and the membrane surface is buckled and irregular
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• marker analysis indicates defective osteoblast-to-osteocyte differentiation and osteocyte maturation
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• osteocyte lacunae are larger and randomly oriented
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• increase in accumulation of trabecular bone in the bone marrow space
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• osteoblasts continuously express osteoblast-specific genes and fail to differentiate into mature osteocytes
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• rachitic rosary of the ribs
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• mice exhibit defects in bone mineralization showing diffuse, osteomalacic form of mineralization; develops with age
• mineral content is either missing or sparsely located in regions surrounding osteocytes
• spherical structures reminiscent of calculospherulites are present
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• mice develop rickets with age, showing, showing flared ends of long bones and rachitic rosary of the ribs
• high-phosphate diet rescues the rickets but not the osteomalacia
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• delay in formation of secondary ossification centers that is mainly due to hypophosphatemia
• mice injected with anti-Fgf23 antibodies show rescue of secondary ossification defects in vertebrae and femoral bone
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• cartilage is remodeled less in 3 day old forelimbs than in controls
• mice at 1 year of age show development of large bony protuberances in all bones, suggesting abnormal bone remodeling processes with age
• bone remodeling abnormalities are due to altered osteoblast function and a reduction of the number of osteoclasts
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hematopoietic system
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• the number of osteoclasts formed in bone marrow cultures in the presence of 1,25-(OH)2D3 is less than 50% of that in control
• marker analysis indicates a reduction in osteoclasts in long bone in both the epiphysis and metaphysis at 2 weeks of age
• however, the number of osteoclasts formed by spleen cells is normal, indicating that osteoclast precursors can differentiate normally
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immune system
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• the number of osteoclasts formed in bone marrow cultures in the presence of 1,25-(OH)2D3 is less than 50% of that in control
• marker analysis indicates a reduction in osteoclasts in long bone in both the epiphysis and metaphysis at 2 weeks of age
• however, the number of osteoclasts formed by spleen cells is normal, indicating that osteoclast precursors can differentiate normally
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• delay in bone marrow formation at E11
• mice injected with anti-Fgf23 antibodies show accelerated bone marrow formation in the femur epiphysis
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Allelic
Composition |
Dmp1tm1Mis/Dmp1tm1.1Mis
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Genetic
Background |
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6 * CBA |
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmp1tm1.1Mis mutation
(0 available);
any
Dmp1 mutation
(42 available)
Dmp1tm1Mis mutation
(0 available);
any
Dmp1 mutation
(42 available)
|
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|
skeleton
|
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• maturation of woven bone is defective
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• secondary ossification is delayed
|
Analysis Tools