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We present a mutation in the ribosomal protein <jats:italic>Rpl27a<\/jats:italic> gene (sooty foot ataxia mice), isolated through a sensitized <jats:italic>N<\/jats:italic>\u2010ethyl\u2010<jats:italic>N<\/jats:italic>\u2010nitrosourea (ENU) mutagenesis screen for p53 pathway defects, that shares striking phenotypic similarities with high <jats:italic>p53<\/jats:italic> mouse models, including cerebellar ataxia, pancytopenia and epidermal hyperpigmentation. This phenocopy is rescued in a haploinsufficient <jats:italic>p53<\/jats:italic> background. A detailed examination of the bone marrow in these mice identified reduced numbers of haematopoietic stem cells and a p53\u2010dependent c\u2010Kit down\u2010regulation. These studies suggest that reduced Rpl27a increases p53 activity <jats:italic>in vivo<\/jats:italic>, further evident with a delay in tumorigenesis in mutant mice. Taken together, these data demonstrate that Rpl27a plays a crucial role in multiple tissues and that disruption of this ribosomal protein affects both development and transformation. Copyright \u00a9 2011 Pathological Society of Great Britain and Ireland. Published by John Wiley &amp; Sons, Ltd.<\/jats:p>","DOI":"10.1002\/path.2891","type":"journal-article","created":{"date-parts":[[2011,3,10]],"date-time":"2011-03-10T09:24:42Z","timestamp":1299749082000},"page":"540-552","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["<i>Rpl27a<\/i> mutation in the sooty foot ataxia mouse phenocopies high p53 mouse 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