Abstract
Malignant infantile osteopetrosis is a rare and lethal disease characterized by the absence of bone resorption due to inactive osteoclasts (OCLs). Among the murine models of osteopetrosis, the Tcirg1oc/oc mouse is the most resembling to the human pathology. In the majority of patients as in Tcirg1oc/oc mouse, the gene involved is the Tcirg1 gene, encoding the a3 subunit of the vacuolar proton pump. However, to date, no osteoclastic cell lines from osteopetrotic mice are available to facilitate the study of either OCL differentiation in osteopetrosis or the factors involved in the control of Tcirg1 gene expression. Heterozygotes Tcirg1+/oc mice were crossed with p53+/- mice to obtain homozygotes p53-/-Tcirg1oc/oc and p53-/-Tcirg1+/+ animals. The p53-/-Tcirg1oc/oc mice display the same bone and hematological phenotype as the original Tcirg1oc/oc mice. From the bone marrow of these mice, we have derived cell lines named POC-MGoc/oc and POC-MG+/+. These cell lines express standard osteoclastogenic markers and differentiate into OCLs in the presence of RANK-L and M-CSF. Furthermore, both cell lines can be transduced by a lentiviral vector with a high efficiency and without alteration of their OCL differentiation potential. Therefore...Continue Reading
References
Jan 1, 1992·The Journal of Cell Biology·V GatteiM L Brandi
Feb 1, 1985·The American Journal of Anatomy·M F Seifert, S C Marks
Mar 1, 1995·Mechanisms of Ageing and Development·G M Gilad, V H Gilad
Jan 1, 1994·Current Biology : CB·T JacksR A Weinberg
Oct 21, 1994·Science·A E GrigoriadisE F Wagner
Nov 1, 1993·Nature Genetics·M HarveyL A Donehower
Jun 15, 1993·Proceedings of the National Academy of Sciences of the United States of America·T J ChambersM D Noble
May 1, 1996·Bone·W G BeamerD J Baylink
Jan 20, 1997·FEBS Letters·I NakamuraT Suda
Mar 6, 1997·Nature·M M TondraviS L Teitelbaum
Jul 28, 1999·Bone·J M QuinnP M Sexton
Sep 29, 1999·Genes & Development·W C DougallJ Schuh
Mar 1, 2000·Journal of Neuroimmunology·P C CharlesC F Brosnan
Mar 10, 2000·Bone·J C ScimecaG F Carle
Aug 15, 2000·Human Molecular Genetics·U KornakC Kubisch
Aug 22, 2000·The Journal of Pathology·T J Chambers
Oct 21, 2000·Archives of Disease in Childhood·C J Wilson, A Vellodi
Sep 5, 2001·Human Molecular Genetics·C SobacchiA Villa
Jan 5, 2002·Blood·Xu-Ming DaiE Richard Stanley
Aug 22, 2002·Cancer Gene Therapy·Redouane RouasAnne Van den Broeke
Jan 1, 2003·The American Journal of Pathology·Anna TarantaAnna Teti
Jan 29, 2003·Human Mutation·Jean-Claude ScimecaGeorges F Carle
Jul 31, 2004·Leukemia·C Blin-WakkachG F Carle
Citations
Feb 22, 2012·The Journal of Experimental Medicine·Anna MansourClaudine Blin-Wakkach
Oct 22, 2011·Journal of Cellular Physiology·Caroline C MoulineJean-Claude Scimeca
Mar 6, 2010·Journal of Cellular Physiology·F LézotA Berdal