Null mutation of chloride channel 7 (Clcn7) impairs dental root formation but does not affect enamel mineralization

Cell and Tissue Research
J GuoA L Bronckers

Abstract

ClC-7, located in late endosomes and lysosomes, is critical for the function of osteoclasts. Secretion of Cl(-) by the ruffled border of osteoclasts enables H(+) secretion by v-H(+)-ATPases to dissolve bone mineral. Mice lacking ClC-7 show altered lysosomal function that leads to severe lysosomal storage. Maturation ameloblasts are epithelial cells with a ruffled border that secrete Cl(-) as well as endocytose and digest large quantities of enamel matrix proteins during formation of dental enamel. We tested the hypothesis that ClC-7 in maturation ameloblasts is required for intracellular digestion of matrix fragments to complete enamel mineralization. Craniofacial bones and developing teeth in Clcn7(-/-) mice were examined by micro-CT, immunohistochemistry, quantified histomorphometry and electron microscopy. Osteoclasts and ameloblasts in wild-type mice stained intensely with anti-ClC-7 antibody but not in Clcn7(-/-) mice. Craniofacial bones in Clcn7(-/-) mice were severely osteopetrotic and contained 1.4- to 1.6-fold more bone volume, which was less mineralized than the wild-type littermates. In Clcn7(-/-) mice maturation ameloblasts and osteoclasts highly expressed Ae2 as in wild-type mice. However, teeth failed to erupt, in...Continue Reading

References

Jun 1, 1989·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·A H SalamaD R Eisenmann
Jul 1, 1994·Calcified Tissue International·H M LinH Ozawa
Apr 1, 1996·Journal of Dental Research·J T WrightB R Grubb
Jun 16, 1999·Calcified Tissue International·J C TiffeeB F Boyce
Sep 22, 1999·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·M GowenI Kola
Jun 5, 2004·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Sophie SchallerMorten A Karsdal
Nov 24, 2004·Journal of Dental Research·J D BartlettC E Smith
Dec 31, 2004·The New England Journal of Medicine·Jakub TolarPaul J Orchard
Feb 12, 2005·The EMBO Journal·Dagmar KasperThomas J Jentsch
May 26, 2006·The New England Journal of Medicine·Ego Seeman, Pierre D Delmas
Dec 14, 2006·The Journal of Clinical Endocrinology and Metabolism·Steven G WaguespackMichael J Econs
Oct 16, 2007·Bone·Andrea Del FattoreAnna Teti
Nov 29, 2007·Matrix Biology : Journal of the International Society for Matrix Biology·D M LyaruuV Everts
Dec 18, 2007·Annals of the New York Academy of Sciences·Haibo Zhao, F Patrick Ross
Mar 1, 2008·Critical Reviews in Biochemistry and Molecular Biology·Thomas J Jentsch
Oct 30, 2008·Calcified Tissue International·Anita Vibsig Neutzsky-WulffKim Henriksen
Nov 7, 2008·Human Genetics·T Segovia-SilvestreK Henriksen
Feb 12, 2009·Journal of Experimental Zoology. Part B, Molecular and Developmental Evolution·Antonius L J J BronckersVincent Everts
May 26, 2009·Biochimica Et Biophysica Acta·Vincent EvertsMiep H Helfrich
Jul 1, 2009·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Ineke D C JansenRonald P J Oude Elferink
Jul 7, 2009·The Journal of Biological Chemistry·James P SimmerJan C-C Hu
Feb 4, 2010·European Journal of Oral Sciences·Xincheng LuJian Q Feng
Jun 23, 2010·The Journal of Cell Biology·Benjamin E SteinbergSergio Grinstein
Oct 4, 2011·Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery·Yang XueXiaohong Duan
Oct 10, 2012·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Rodrigo S LacruzMichael L Paine
Feb 22, 2013·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Antonius L J J BronckersBernd Schröder
Oct 11, 2013·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Florian BarvencikMichael Amling
Feb 11, 2015·The Anatomical Record : Advances in Integrative Anatomy and Evolutionary Biology·Xin WenMichael L Paine

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Citations

May 5, 2017·Physiological Reviews·Rodrigo S LacruzMichael L Paine
May 31, 2018·Physiological Reviews·Thomas J Jentsch, Michael Pusch
Nov 8, 2018·Experimental and Therapeutic Medicine·Mei JiDongsheng Zhang

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