In vitro differentiation of CD14 cells from osteopetrotic subjects: contrasting phenotypes with TCIRG1, CLCN7, and attachment defects

Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research
Harry C BlairPaul J Orchard

Abstract

We studied osteoclastic differentiation from normal and osteopetrotic human CD14 cells in vitro. Defects in acid transport, organic matrix removal, and cell fusion with deficient attachment were found. Analysis of genotypes showed that TCIRG1 anomalies correlated with acid transport defects, but surprisingly, organic matrix removal failure correlated with CLCN7 defects; an attachment defect had normal TCIRG1 and CLCN7. Osteopetrotic subjects usually have normal macrophage activity, and despite identification of genetic defects associated with osteopetrosis, the specific developmental and biochemical defects in most cases are unclear. Indeed, patients with identical genotypes often have different clinical courses. We classified defects in osteoclast differentiation in vitro using four osteopetrotic subjects without immune or platelet defects, three of them severe infantile cases, compared with normals. Osteoclast differentiation used isolated CD14 cells; results were correlated with independent analysis of two key genes, CLCN7 and TCIRG1. CD14 cell attachment and cell surface markers and extent of differentiation in RANKL and colony-stimulating factor (CSF)-1 were studied using acid secretion, bone pitting, enzyme, and attachmen...Continue Reading

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Citations

Oct 1, 2005·Proceedings of the National Academy of Sciences of the United States of America·Annalisa FrattiniAnna Villa
Nov 25, 2005·Calcified Tissue International·W BalemansW Van Hul
Jul 13, 2006·The Journal of Biological Chemistry·John C EdwardsPaul H Schlesinger
Oct 30, 2008·Calcified Tissue International·Anita Vibsig Neutzsky-WulffKim Henriksen
Dec 17, 2008·Calcified Tissue International·Anna VillaCristina Sobacchi
Jul 3, 2010·Calcified Tissue International·Jonas K E Nyman, H Kalervo Väänänen
Nov 23, 2006·Reviews in Endocrine & Metabolic Disorders·Harry C Blair, Mone Zaidi
Nov 7, 2008·Human Genetics·T Segovia-SilvestreK Henriksen
Dec 17, 2008·Biochemical and Biophysical Research Communications·Kim HenriksenMorten A Karsdal
Sep 5, 2008·Seminars in Cell & Developmental Biology·Fraser P Coxon, Adam Taylor
Oct 16, 2007·Bone·Andrea Del FattoreAnna Teti
Jul 4, 2006·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Kang ChuMichael J Econs
Jun 16, 2011·BioFactors·Harry C BlairMone Zaidi
Jul 9, 2008·American Journal of Human Genetics·Matteo M GuerriniAnnalisa Frattini
Apr 25, 2006·Cell·Li SunMone Zaidi
Mar 21, 2017·The Journal of Endocrinology·Nicholaos I PapachristouDionysios J Papachristou
Jul 24, 2013·Nature Reviews. Endocrinology·Cristina SobacchiMiep H Helfrich
Jun 24, 2009·Laboratory Investigation; a Journal of Technical Methods and Pathology·Harry C BlairPaul J Orchard
Nov 18, 2005·Journal of Cell Science·Beatrice B YaroslavskiyHarry C Blair

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