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The exact cause of these interhemispheric lipomas is not known, but they arise from a developmental defect resulting in the maldifferentiation of mesenchymal cells into mesodermal derivatives that are not normally present. We have identified a new mouse mutant called <jats:italic>tuft<\/jats:italic>, exhibiting a forebrain, intracranial lipoma with midline craniofacial defects resembling frontonasal dysplasia (FND) that arose spontaneously in our wild\u2010type 3H1 colony. The <jats:italic>tuft<\/jats:italic> trait seems to be transmitted in recessive fashion, but approximately 80% less frequent than the expected Mendelian 25%, due to either incomplete penetrance or prenatal lethality. MRI and histologic analysis revealed that the intracranial lipoma occurred between the hemispheres and often protruded through the sagittal suture. We also observed a lesion at the lamina terminalis (LT) that may indicate improper closure of the anterior neuropore. We have mapped the <jats:italic>tuft<\/jats:italic> trait to within an 18 cM region on mouse chromosome 10 by microsatellite linkage analysis and identified several candidate genes involved with craniofacial development and cellular differentiation of adipose tissue. <jats:italic>Tuft<\/jats:italic> is the only known mouse model for midline craniofacial defects with an intracranial lipoma. Identifying the gene(s) and mutation(s) causing this early developmental defect will help us understand the pathogenesis of FND and related craniofacial disorders. \u00a9 Birth Defects Research (Part A), 2012. \u00a9 2012 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/bdra.22878","type":"journal-article","created":{"date-parts":[[2012,1,13]],"date-time":"2012-01-13T14:38:45Z","timestamp":1326465525000},"page":"102-113","source":"Crossref","is-referenced-by-count":3,"title":["Craniofacial features resembling frontonasal dysplasia with a tubulonodular interhemispheric lipoma in the adult 3H1 <i>tuft<\/i> mouse"],"prefix":"10.1002","volume":"94","author":[{"given":"Keith S. 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