Developmental arrest of angioblastic lineage initiates tumorigenesis in von Hippel-Lindau disease.
نویسندگان
چکیده
The nature of the cell responsible for von Hippel-Lindau (VHL) disease-associated tumor formation has been controversial for decades. We demonstrate that VHL disease-associated central nervous system tumors are composed of developmentally arrested angioblasts that coexpress erythropoietin (Epo) and Epo receptor. The angioblasts are capable of differentiating into RBCs via formation of blood islands with extramedullary hematopoiesis. Because of VHL deficiency, Epo receptor-expressing, developmentally arrested angioblasts simultaneously coexpress Epo, which may represent a crucial pathogenetic step in tumor formation.
منابع مشابه
Tumorigenesis in von Hippel-Lindau Disease Developmental Arrest of Angioblastic Lineage Initiates
The nature of the cell responsible for von Hippel-Lindau (VHL) disease-associated tumor formation has been controversial for decades. We demonstrate that VHL disease-associated central nervous system tumors are composed of developmentally arrested angioblasts that coexpress erythropoietin (Epo) and Epo receptor. The angioblasts are capable of differentiating into RBCs via formation of blood isl...
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ورودعنوان ژورنال:
- Cancer research
دوره 63 21 شماره
صفحات -
تاریخ انتشار 2003