title: Developmental arrest of angioblastic lineage initiates tumorigenesis in von Hippel-Lindau disease.
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.
authors: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke/NIH, 10 Center Drive, Bethesda, MD 20892, USA.
authors: Alexander O
authors: AO
authors: Vortmeyer
authors: Stephan
authors: S
authors: Frank
authors: Seon-Yong
authors: SY
authors: Jeong
authors: Kristy
authors: K
authors: Yuan
authors: Barbara
authors: B
authors: Ikejiri
authors: Youn-Soo
authors: YS
authors: Lee
authors: Deb
authors: D
authors: Bhowmick
authors: Russell R
authors: RR
authors: Lonser
authors: Reginald
authors: R
authors: Smith
authors: Griffin
authors: G
authors: Rodgers
authors: Edward H
authors: EH
authors: Oldfield
authors: Zhengping
authors: Z
authors: Zhuang
mesh_terms: D002528:Cerebellar Neoplasms / Q000209:etiology* / Q000378:metabolism / Q000473:pathology*; D004921:Erythropoietin / Q000096:biosynthesis / Q000378:metabolism; D018325:Hemangioblastoma / Q000209:etiology* / Q000378:metabolism / Q000473:pathology*; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D007150:Immunohistochemistry; D017467:Receptors, Erythropoietin / Q000096:biosynthesis / Q000378:metabolism; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000235:genetics / Q000473:pathology*
chemical_list: D017467:Receptors, Erythropoietin; D004921:Erythropoietin