Developmental stage-specific proliferation and retinoblastoma genesis in RB-deficient human but not mouse cone precursors.

ABSTRACT: Most retinoblastomas initiate in response to the inactivation of the RB1 gene and loss of functional RB protein. The tumors may form with few additional genomic changes and develop after a premalignant retinoma phase. Despite this seemingly straightforward etiology, mouse models have not recapitulated the genetic, cellular, and stage-specific features of human retinoblastoma genesis. For example, whereas human retinoblastomas appear to derive from cone photoreceptor precursors, current mouse models develop tumors that derive from other retinal cell types. To investigate the basis of the human cone-specific oncogenesis, we compared developmental stage-specific cone precursor responses to RB loss in human and murine retina cultures and in cone-specific Rb1-knockout mice. We rep

SUBMITTER: Singh HP 

PROVIDER: S-EPMC6176579 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature