Developmental stage-specific proliferation and retinoblastoma genesis in RB-deficient human but not mouse cone precursors
نویسندگان
چکیده
منابع مشابه
Retinoblastoma Has Properties of a Cone Precursor Tumor and Depends Upon Cone-Specific MDM2 Signaling
Retinoblastomas result from the inactivation of the RB1 gene and the loss of Rb protein, yet the cell type in which Rb suppresses retinoblastoma and the circuitry that underlies the need for Rb are undefined. Here, we show that retinoblastoma cells express markers of postmitotic cone precursors but not markers of other retinal cell types. We also demonstrate that human cone precursors prominent...
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We recently found that the Rb protein is important for the regulation of retinal progenitor cell proliferation and rod photoreceptor development in the mouse retina. These two functions are separate for Rb and in this study we further characterize the role of Rb in retinal development. At postnatal day 12 in the retinae of Chx10-Cre;RbLox/- mice, immature cells are found in the outer nuclear la...
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PURPOSE The effects of postnatal hypothyroidism on retinal development and spatial patterning of cone opsin expression were studied in Pax8-deficient mice. Pax8(-/-) mice are incapable of synthesizing thyroxine and serve as a model for congenital hypothyroidism. METHODS Pax8(-/-), Pax8(+/-), and Pax8(+/+) littermates were studied. Serum thyroid hormone levels, body weight, and eye size were m...
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Retinoblastoma is a pediatric cancer that has served as a paradigm for tumor suppressor gene function. Retinoblastoma is initiated by RB gene mutations, but the subsequent cooperating mutational events leading to tumorigenesis are poorly characterized. We investigated what these additional genomic alterations might be using human retinoblastoma samples and mouse models. Array-based comparative ...
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Mutations of the survival of motor neuron gene (SMN1) are responsible for spinal muscular atrophies (SMA), a frequent recessive autosomal motor neuron disease. SMN is involved in various processes including RNA metabolism. However, the molecular pathway linking marked deficiency of SMN to SMA phenotype remains unclear. Homozygous deletion of murine Smn exon 7 directed to neurons or skeletal mus...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2018
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.1808903115