Ontology highlight
ABSTRACT:
SUBMITTER: Moisan A
PROVIDER: S-EPMC4052985 | biostudies-literature | 2011 May
REPOSITORIES: biostudies-literature
Moisan Annie A Rivera Miguel N MN Lotinun Sutada S Akhavanfard Sara S Coffman Erik J EJ Cook Edward B EB Stoykova Svetlana S Mukherjee Siddhartha S Schoonmaker Jesse A JA Burger Alexa A Kim Woo Jae WJ Kronenberg Henry M HM Baron Roland R Haber Daniel A DA Bardeesy Nabeel N
Developmental cell 20110501 5
WTX is an X-linked tumor suppressor targeted by somatic mutations in Wilms tumor, a pediatric kidney cancer, and by germline inactivation in osteopathia striata with cranial sclerosis, a bone overgrowth syndrome. Here, we show that Wtx deletion in mice causes neonatal lethality, somatic overgrowth, and malformation of multiple mesenchyme-derived tissues, including bone, fat, kidney, heart, and spleen. Inactivation of Wtx at different developmental stages and in primary mesenchymal progenitor cel ...[more]