Developmental arrest of angioblastic lineage initiates tumorigenesis in von Hippel-Lindau disease.
Cancer Res
; 63(21): 7051-5, 2003 Nov 01.
Article
em En
| MEDLINE
| ID: mdl-14612494
ABSTRACT
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Cerebelares
/
Hemangioblastoma
/
Doença de von Hippel-Lindau
Limite:
Humans
Idioma:
En
Ano de publicação:
2003
Tipo de documento:
Article