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The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells
- Source :
- Bronckers, A, Kalogeraki, L, Jorna, H J N, Wilke, M, Bervoets, T J, Lyaruu, D M, Zandieh Doulabi, B, DenBesten, P & de Jonge, H 2010, ' The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells ', Bone, vol. 46, no. 4, pp. 1188-1196 . https://doi.org/10.1016/j.bone.2009.12.002, Bone, 46(4), 1188-1196. Elsevier Inc., Bronckers, A, Kalogeraki, L, Jorna, H J N, Wilke, M, Bervoets, T J, Lyaruu, D M, Zandieh-Doulabi, B, Denbesten, P & de Jonge, H 2010, ' The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells ', Bone, vol. 46, no. 4, pp. 1188-1196 . https://doi.org/10.1016/j.bone.2009.12.002, Bone, 46(4), 1188-1196. Elsevier
- Publication Year :
- 2010
-
Abstract
- Patients with cystic fibrosis (CF) have mild defects in dental enamel. The gene mutated in these patients is CFTR, a Cl- channel involved in transepithelial salt and water transport and bicarbonate secretion. We tested the hypothesis that Cftr channels are present and operating in the plasma membranes of mouse ameloblasts. Tissue sections of young mouse jaws and fetal human jaws were immunostained with various anti-Cftr antibodies. Specificity of the antibodies was validated in Cftr-deficient murine and human tissues. Immunostaining for Cftr was obtained in the apical plasma membranes of mouse maturation ameloblasts of both incisor and molar tooth germs. A granular intracellular immunostaining of variable intensity was also noted in bone cells and odontoblasts. In Cftr-deficient mice the incisors were chalky white and eroded much faster than in wild type mice. Histologically, only maturation ameloblasts of incisors were structurally affected in Cftr-deficient mice. Some antibody species gave also a positive cytosolic staining in Cftr-deficient cells. Transcripts of Cftr were found in maturation ameloblasts, odontoblasts and bone cells. Similar data were obtained in forming human dentin and bone. We conclude that Cftr protein locates in the apical plasma membranes of mouse maturation ameloblasts. In mouse incisors Cftr is critical for completion of enamel mineralization and conceivably functions as a regulator of pH during rapid crystal growth. Osteopenia found in CF patients as well as in Cftr-deficient mice is likely associated with defective Cftr operating in bone cells. (C) 2009 Elsevier Inc. All rights reserved.
- Subjects :
- Pathology
medicine.medical_specialty
congenital, hereditary, and neonatal diseases and abnormalities
Histology
Physiology
Endocrinology, Diabetes and Metabolism
Cystic Fibrosis Transmembrane Conductance Regulator
Osteocytes
Article
Mice
stomatognathic system
Bone cell
medicine
Ameloblasts
Animals
Humans
Dental Enamel
Mice, Knockout
Water transport
biology
Odontoblasts
Chemistry
Reverse Transcriptase Polymerase Chain Reaction
Osteoblast
respiratory system
Immunohistochemistry
Cystic fibrosis transmembrane conductance regulator
Cell biology
respiratory tract diseases
Incisor
Enamel mineralization
stomatognathic diseases
Odontoblast
medicine.anatomical_structure
Jaw
Dentin
biology.protein
Odontogenesis
Ameloblast
Immunostaining
Subjects
Details
- ISSN :
- 87563282
- Database :
- OpenAIRE
- Journal :
- Bronckers, A, Kalogeraki, L, Jorna, H J N, Wilke, M, Bervoets, T J, Lyaruu, D M, Zandieh Doulabi, B, DenBesten, P & de Jonge, H 2010, ' The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells ', Bone, vol. 46, no. 4, pp. 1188-1196 . https://doi.org/10.1016/j.bone.2009.12.002, Bone, 46(4), 1188-1196. Elsevier Inc., Bronckers, A, Kalogeraki, L, Jorna, H J N, Wilke, M, Bervoets, T J, Lyaruu, D M, Zandieh-Doulabi, B, Denbesten, P & de Jonge, H 2010, ' The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells ', Bone, vol. 46, no. 4, pp. 1188-1196 . https://doi.org/10.1016/j.bone.2009.12.002, Bone, 46(4), 1188-1196. Elsevier
- Accession number :
- edsair.doi.dedup.....758f70eeb87d590090eb029e1b84dceb