crx is a diagnostic marker of retinal and pineal lineage tumorscrx是视网膜的诊断标记和松果体家族肿瘤.pdfVIP

CRX Is a Diagnostic Marker of Retinal and Pineal Lineage Tumors 1,2,3 3,4 3,4 5 6 6 Sandro Santagata , Cecile L. Maire , Ahmed Idbaih , Lars Geffers , Mick Correll , Kristina Holton , John Quackenbush6, Keith L. Ligon1,2,3,4* 1 Department of Pathology, Brigham and Women’s Hospital, Boston, Massachusetts, United States of America, 2 Department of Pathology, Children’s Hospital Boston, Boston, Massachusetts, United States of America, 3 Department of Pathology, Harvard Medical School, Boston, Massachusetts, United States of America, 4 Department of Medical Oncology and Center for Molecular Oncologic Pathology, Dana-Farber Cancer Institute, Boston, Massachusetts, United States of America, 5 Department of Genes and Behavior, Max-Planck-Institute of Biophysical Chemistry, Goettingen, Germany, 6 Center for Cancer Computational Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, United States of America Abstract Background: CRX is a homeobox transcription factor whose expression and function is critical to maintain retinal and pineal lineage cells and their progenitors. To determine the biologic and diagnostic potential of CRX in human tumors of the retina and pineal, we examined its expression in multiple settings. Methodology/Principal Findings: Using situ hybridization and immunohistochemistry we show that Crx RNA and protein expression are exquisitely lineage restricted to retinal and pineal cells during normal mouse and human development. Gene expression profiling analysis of a wide range of human cancers and cancer cell lines also supports that CRX RNA is highly lineage restricted in cancer. Immunohistochemical analysis of 22 retinoblastomas and 13 pineal parenchymal tumors demonst