Article
Loss of ATRX or DAXX expression and concomitant acquisition of the alternative lengthening of telomeres phenotype are late events in a small subset of MEN-1 syndrome pancreatic neuroendocrine tumors.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc - 1 Jul 2012
de Wilde Roeland F, Heaphy Christopher M, Maitra Anirban, Meeker Alan K, Edil Barish H, Wolfgang Christopher L, Ellison Trevor A, Schulick Richard D, Molenaar I Quintus, Valk Gerlof D, Vriens Menno R, Borel Rinkes Inne H M, Offerhaus G Johan A, Hruban Ralph H, Matsukuma Karen E
Abstract excerpt
Approximately 45% of sporadic well-differentiated pancreatic neuroendocrine tumors harbor mutations in either ATRX (alpha thalassemia/mental retardation X-linked) or DAXX (death domain-associated protein). These novel tumor suppressor genes encode nuclear proteins that interact with one another and function in chromatin remodeling at telomeric and peri-centromeric regions. Mutations in these genes are associated...
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