Abstract
ClC-7 is a 2Cl<sup>-</sup>/1H<sup>+</sup>-exchanger expressed at late endosomes and lysosomes, as well as the ruffled border of osteoclasts. ClC-7 deficiencies in mice and humans lead to impaired osteoclast function and therefore osteopetrosis. Failure of tooth eruption is also apparent in ClC-7 mutant animals, and this has been attributed to the osteoclast dysfunction and the subsequent defect in alveolar bone resorptive activity surrounding tooth roots. Ameloblasts also express ClC-7, and this study aims to determine the significance of ClC-7 in enamel formation by examining the dentitions of ClC-7 mutant mice. Micro-CT analysis revealed that the molar teeth of 3-week old ClC-7 mutant mice had no roots, and the incisors were smaller than their age-matched controls. Despite these notable developmental differences, the enamel and dentin densities of the mutant mice were comparable to those of the wild-type littermates. Scanning electron microscopy showed normal enamel crystallite and prismatic organization in the ClC-7 mutant mice, although the enamel was thinner (hypoplastic) than in controls. These results suggested that ClC-7 was not critical to enamel and dentin formation, and the observed tooth defects may be related more to a resulting alveolar bone phenotype. Micro-CT analysis also revealed abnormal features in the calvarial bones of the mutant mice. The cranial sutures in ClC-7 mutant mice remained open compared to the closed sutures seen in the control mice at 3 weeks. These data demonstrate that ClC-7 deficiency impacts the development of the dentition and calvaria, but does not significantly disrupt amelogenesis.
Original language | English (US) |
---|---|
Pages (from-to) | 1502-1508 |
Number of pages | 7 |
Journal | Anatomical Record |
Volume | 298 |
Issue number | 8 |
DOIs | |
State | Published - Aug 1 2015 |
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Keywords
- Ameloblast
- Amelogenesis
- Biomineralization
- Chloride channels
- Craniofacial development
- Enamel
- PH regulation
ASJC Scopus subject areas
- Anatomy
- Histology
- Ecology, Evolution, Behavior and Systematics
- Biotechnology
Cite this
Dental and Cranial Pathologies in Mice Lacking the Cl<sup>-</sup>/H<sup>+</sup>-Exchanger ClC-7. / Wen, Xin; Lacruz, Rodrigo; Paine, Michael L.
In: Anatomical Record, Vol. 298, No. 8, 01.08.2015, p. 1502-1508.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Dental and Cranial Pathologies in Mice Lacking the Cl-/H+-Exchanger ClC-7
AU - Wen, Xin
AU - Lacruz, Rodrigo
AU - Paine, Michael L.
PY - 2015/8/1
Y1 - 2015/8/1
N2 - ClC-7 is a 2Cl-/1H+-exchanger expressed at late endosomes and lysosomes, as well as the ruffled border of osteoclasts. ClC-7 deficiencies in mice and humans lead to impaired osteoclast function and therefore osteopetrosis. Failure of tooth eruption is also apparent in ClC-7 mutant animals, and this has been attributed to the osteoclast dysfunction and the subsequent defect in alveolar bone resorptive activity surrounding tooth roots. Ameloblasts also express ClC-7, and this study aims to determine the significance of ClC-7 in enamel formation by examining the dentitions of ClC-7 mutant mice. Micro-CT analysis revealed that the molar teeth of 3-week old ClC-7 mutant mice had no roots, and the incisors were smaller than their age-matched controls. Despite these notable developmental differences, the enamel and dentin densities of the mutant mice were comparable to those of the wild-type littermates. Scanning electron microscopy showed normal enamel crystallite and prismatic organization in the ClC-7 mutant mice, although the enamel was thinner (hypoplastic) than in controls. These results suggested that ClC-7 was not critical to enamel and dentin formation, and the observed tooth defects may be related more to a resulting alveolar bone phenotype. Micro-CT analysis also revealed abnormal features in the calvarial bones of the mutant mice. The cranial sutures in ClC-7 mutant mice remained open compared to the closed sutures seen in the control mice at 3 weeks. These data demonstrate that ClC-7 deficiency impacts the development of the dentition and calvaria, but does not significantly disrupt amelogenesis.
AB - ClC-7 is a 2Cl-/1H+-exchanger expressed at late endosomes and lysosomes, as well as the ruffled border of osteoclasts. ClC-7 deficiencies in mice and humans lead to impaired osteoclast function and therefore osteopetrosis. Failure of tooth eruption is also apparent in ClC-7 mutant animals, and this has been attributed to the osteoclast dysfunction and the subsequent defect in alveolar bone resorptive activity surrounding tooth roots. Ameloblasts also express ClC-7, and this study aims to determine the significance of ClC-7 in enamel formation by examining the dentitions of ClC-7 mutant mice. Micro-CT analysis revealed that the molar teeth of 3-week old ClC-7 mutant mice had no roots, and the incisors were smaller than their age-matched controls. Despite these notable developmental differences, the enamel and dentin densities of the mutant mice were comparable to those of the wild-type littermates. Scanning electron microscopy showed normal enamel crystallite and prismatic organization in the ClC-7 mutant mice, although the enamel was thinner (hypoplastic) than in controls. These results suggested that ClC-7 was not critical to enamel and dentin formation, and the observed tooth defects may be related more to a resulting alveolar bone phenotype. Micro-CT analysis also revealed abnormal features in the calvarial bones of the mutant mice. The cranial sutures in ClC-7 mutant mice remained open compared to the closed sutures seen in the control mice at 3 weeks. These data demonstrate that ClC-7 deficiency impacts the development of the dentition and calvaria, but does not significantly disrupt amelogenesis.
KW - Ameloblast
KW - Amelogenesis
KW - Biomineralization
KW - Chloride channels
KW - Craniofacial development
KW - Enamel
KW - PH regulation
UR - http://www.scopus.com/inward/record.url?scp=84936985911&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84936985911&partnerID=8YFLogxK
U2 - 10.1002/ar.23118
DO - 10.1002/ar.23118
M3 - Article
C2 - 25663454
AN - SCOPUS:84936985911
VL - 298
SP - 1502
EP - 1508
JO - Anatomical Record
JF - Anatomical Record
SN - 1932-8486
IS - 8
ER -