CRAC channels in dental enamel cells |
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| Affiliation: | 1. Department of Vegetative Physiology, Eberhad Karls University, Wilhelmstr. 56, D-72074 Tübingen, Germany;2. German Center for Neurodegenerative Diseases, Research Site Tübingen, Germany;3. Department of Neurology and Hertie Institute for Clinical Brain Research, University of Tübingen, Germany;4. Department of Neurology and Center for Regenerative Therapies Dresden (CRTD), Technische Universität Dresden, Germany & DZNE, German Center for Neurodegenerative Diseases, Research Site Dresden, Germany;5. Department of Biochemistry, University of Crete Medical School, Heraklion, Greece;1. Laboratory of Skin Biology, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA;2. Pachyonychia Congenita Project, Holladay, Utah, USA;1. Department of Dental Hygiene, College of Health Science, Gachon University, 191 Hambangmoe-ro, Yeonsu-gu, Incheon, 21936, South Korea;2. Department of Physiology, College of Medicine, Lee Gil Ya Cancer and Diabetes Institute, GAIHST, Gachon University, Incheon, 21999, South Korea;1. Department of Medical Genetics, Oslo University Hospital and University of Oslo, Oslo, Norway;2. Faculty of Veterinary Medicine, Norwegian University of Life Sciences, Norway;3. Department of Orthodontics, Institute of Clinical Dentistry, University of Oslo, Oslo, Norway;4. Department of Biomaterials, Institute of Clinical Dentistry, University of Oslo, Oslo, Norway;5. Department of Radiology and Nuclear Medicine, Oslo University Hospital, Oslo, Norway;6. Neurology Unit, Fondazione IRCCS Ca'' Granda Ospedale Maggiore Policlinico, Milan, Italy;7. Neuroscience Section, Department of Pathophysiology and Transplantation, Dino Ferrari Centre, University of Milan, Milan, Italy;8. Department of Neurology, Nakamura Memorial Hospital, Sapporo, Japan;9. CHRU, Hôpital Gui de Chauliac, Montpellier, France;10. Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma, USA;11. Department of Haematology, Oslo University Hospital and Institute of Clinical Medicine, University of Oslo, Oslo, Norway;12. Department of Basic Science and Craniofacial Biology, New York University College of Dentistry, New York, USA |
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| Abstract: | Enamel mineralization relies on Ca2+ availability provided by Ca2+ release activated Ca2+ (CRAC) channels. CRAC channels are modulated by the endoplasmic reticulum Ca2+ sensor STIM1 which gates the pore subunit of the channel known as ORAI1, found the in plasma membrane, to enable sustained Ca2+ influx. Mutations in the STIM1 and ORAI1 genes result in CRAC channelopathy, an ensemble of diseases including immunodeficiency, muscular hypotonia, ectodermal dysplasia with defects in sweat gland function and abnormal enamel mineralization similar to amelogenesis imperfecta (AI). In some reports, the chief medical complain has been the patient’s dental health, highlighting the direct and important link between CRAC channels and enamel. The reported enamel defects are apparent in both the deciduous and in permanent teeth and often require extensive dental treatment to provide the patient with a functional dentition. Among the dental phenotypes observed in the patients, discoloration, increased wear, hypoplasias (thinning of enamel) and chipping has been reported. These findings are not universal in all patients. Here we review the mutations in STIM1 and ORAI1 causing AI-like phenotype, and evaluate the enamel defects in CRAC channel deficient mice. We also provide a brief overview of the role of CRAC channels in other mineralizing systems such as dentine and bone. |
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| Keywords: | Enamel CRAC channels Mutations Amelogenesis imperfecta Hypoplasia |
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