Journal article
Enamel Pathology Resulting from Loss of Function in the Cystic Fibrosis Transmembrane Conductance Regulator in a Porcine Animal Model
Cells, tissues, organs, Vol.194(2-4), pp.249-254
08/2011
DOI: 10.1159/000324248
PMCID: PMC3178086
PMID: 21525720
Abstract
Cystic fibrosis (CF) is caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), a phosphorylation- and ATP-regulated anion channel. CFTR expression and activity is frequently associated with an anion exchanger (AE) such as AE2 coded by the
Slc4a2
gene. Mice null for
Cftr
and mice null for
Slc4a2
have enamel defects, and there are some case reports of enamel anomalies in patients with CF. In this study we demonstrate that both Cftr and AE2 expression increased significantly during the rat enamel maturation stage versus the earlier secretory stage (5.6- and 2.9-fold, respectively). These qPCR data im- ply that there is a greater demand for Cl
–
and bicarbonate (HCO
3
–
) transport during the maturation stage of enamel formation, and that this is, at least in part, provided by changes in Cftr and AE2 expression. In addition, the enamel phenotypes of 2 porcine models of CF,
CFTR-
null, and
CFTR-
ΔF508 have been examined using backscattered electron microscopy in a scanning electron microscope. The enamel of newborn
CFTR-
null and
CFTR-
ΔF508 animals is hypomineralized. Together, these data provide a molecular basis for interpreting enamel disease associated with disruptions to CFTR and AE2 expression.
Details
- Title: Subtitle
- Enamel Pathology Resulting from Loss of Function in the Cystic Fibrosis Transmembrane Conductance Regulator in a Porcine Animal Model
- Creators
- Eugene H Chang - Department of Otolaryngology, University of Iowa, Iowa City, IowaRodrigo S Lacruz - Center for Craniofacial and Molecular Biology, Herman Ostrow School of Dentistry, Los Angeles, CalifTimothy G Bromage - Department of Biomaterials and Biomimetics, New York University College of Dentistry, New York, N.Y., USAPablo Bringas - Center for Craniofacial and Molecular Biology, Herman Ostrow School of Dentistry, Los Angeles, CalifMichael J Welsh - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IowaJoseph Zabner - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IowaMichael L Paine - Center for Craniofacial and Molecular Biology, Herman Ostrow School of Dentistry, Los Angeles, Calif
- Resource Type
- Journal article
- Publication Details
- Cells, tissues, organs, Vol.194(2-4), pp.249-254
- Publisher
- S. Karger AG
- DOI
- 10.1159/000324248
- PMID
- 21525720
- PMCID
- PMC3178086
- eISBN
- 9783805598422; 3805598424
- ISSN
- 1422-6405
- eISSN
- 1422-6421
- Language
- English
- Date published
- 08/2011
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Anatomy and Cell Biology; Neurosurgery; Internal Medicine
- Record Identifier
- 9984013920302771
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