Regular Article
Retinoblastoma: the disease, gene and protein provide critical leads to understand cancer

https://doi.org/10.1006/scbi.2000.0326Get rights and content

Abstract

Retinoblastoma has contributed much to the understanding of cancer. The protein product of the RB gene, pRB, is a multifaceted regulator of transcription which controls the cell cycle, differentiation and apoptosis in normal development of specific tissues. Elucidating the mechanisms in which pRB plays a critical role will enable novel therapies and strategies for prevention, not only for retinoblastoma, but for cancer in general.

References (179)

  • D Choubey et al.

    Binding of an interferon-inducible protein (p202) to the retinoblastoma protein

    J Biol Chem

    (1995)
  • CJ Fry et al.

    Context-dependent transcriptional regulation

    J Biol Chem

    (1999)
  • JF Ross et al.

    Mechanism of transcriptional repression of E2F by the retinoblastoma tumor suppressor protein

    Mol Cell

    (1999)
  • J Adnane et al.

    The retinoblastoma susceptibility gene product represses transcription when directly bound to the promoter

    J Biol Chem

    (1995)
  • RX Luo et al.

    Rb interacts with histone deacetylase to repress transcription

    Cell

    (1998)
  • YW Qian et al.

    Dual retinoblastoma-binding proteins with properties related to a negative regulator of ras in yeast

    J Biol Chem

    (1995)
  • L Yamasaki et al.

    Tumor induction and tissue atrophy in mice lacking E2F-1

    Cell

    (1996)
  • RA Weinberg

    E2F and cell proliferation: a world turned upside down

    Cell

    (1996)
  • JL Dunaief et al.

    The retinoblastoma protein and BRG1 form a complex and cooperate to induce cell cycle arrest

    Cell

    (1994)
  • Y Zhang et al.

    Histone deacetylases and SAP18, a novel polypeptide, are components of a human Sin3 complex

    Cell

    (1997)
  • JT Kadonaga

    Eukaryotic transcription: an interlaced network of transcription factors and chromatin-modifying machines

    Cell

    (1998)
  • JL Dunaief et al.

    The retinoblastoma protein and BRG1 form a complex and cooperate to induce cell cycle arrest

    Cell

    (1994)
  • HS Zhang et al.

    Exit from G1 and S phase of the cell cycle is regulated by repressor complexes containing HDAC-Rb-hSWI/SNF and Rb-hSWI/SNF

    Cell

    (2000)
  • FC Holstege et al.

    Dissecting the regulatory circuitry of a eukaryotic genome

    Cell

    (1998)
  • C Eng et al.

    Mortality from second tumors among long-term survivors of retinoblastoma

    JNCI

    (1993)
  • RA Phillips et al.

    Why don’t germline mutations in RB1 predispose to leukemia? [Review]

    Curr Top Microbiol Immunol

    (1992)
  • HZ Noorani et al.

    Cost comparison of molecular versus conventional screening of relatives at risk for retinoblastoma [see comments]

    Am J Hum Genet

    (1996)
  • E de Sutter et al.

    The prognosis of retinoblastoma in terms of survival. A computer assisted study. Part II

    Ophthalmic Paediatr Genet

    (1987)
  • AG Knudson

    Mutation and cancer: statistical study of retinoblastoma

    Proc Natl Acad Sci USA

    (1971)
  • DE Comings

    A general theory of carcinogenesis

    Proc Natl Acad Sci USA

    (1973)
  • RS Sparkes et al.

    Regional assignment of genes for human esterase D and retinoblastoma to chromosome band 13q14

    Science (Washington DC)

    (1980)
  • MJ Connolly et al.

    Familial, EsD-linked, retinoblastoma with reduced penetrance and variable expressivity

    Hum Genet

    (1983)
  • JJ Yunis et al.

    Retinoblastoma and subband deletion of chromosome 13

    Am J Dis Child

    (1978)
  • R Godbout et al.

    Somatic inactivation of genes on chromosome 13 is a common event in retinoblastoma

    Nature (London)

    (1983)
  • WK Cavenee et al.

    Expression of recessive alleles by chromosomal mechanisms in retinoblastoma

    Nature (London)

    (1983)
  • X Zhu et al.

    Mechanisms of loss of heterozygosity in retinoblastoma

    Cytogenet Cell Genet

    (1992)
  • TP Dryja et al.

    Molecular detection of deletions involving band q14 of chromosome 13 in retinoblastomas

    Proc Natl Acad Sci USA

    (1986)
  • SH Friend et al.

    A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma

    Nature (London)

    (1986)
  • JM Dunn et al.

    Identification of germline and somatic mutations affecting the retinoblastoma gene

    Science (Washington DC)

    (1988)
  • E Robanus-Maandag et al.

    p107 is a suppressor of retinoblastoma development in pRb-deficient mice

    Genes Dev

    (1998)
  • T Jacks et al.

    Effects of an Rb mutation in the mouse

    Nature (London)

    (1992)
  • EH-HP Lee et al.

    Mice deficient for Rb are nonviable and show defects in neurogenesis and haematopoiesis

    Nature (London)

    (1992)
  • AR Clarke et al.

    Requirement for a functional Rb-1 gene in murine development

    Nature

    (1992)
  • BL Gallie et al.

    Retinoma: spontaneous regression of retinoblastoma or benign manifestation of the mutation?

    Br J Cancer

    (1982)
  • DH Abramson

    Retinoma, retinocytoma, and the retinoblastoma gene [editorial]

    Arch Ophthalmol

    (1983)
  • VR Potluri et al.

    Chromosomal abnormalities in human retinoblastoma. A review

    Cancer

    (1986)
  • J Squire et al.

    A detailed analysis of chromosomal changes in heritable and non-heritable retinoblastoma

    Hum Genet

    (1985)
  • LE Kusnetsova et al.

    Similar chromosomal abnormalities in several retinoblastomas

    Hum Genet

    (1982)
  • J Squire et al.

    Isochromosome 6p, a unique chromosomal abnormality in retinoblastoma: verification by standard staining techniques, new densitometric methods, and somatic cell hybridization

    Hum Genet

    (1984)
  • BL Gallie et al.

    Developmental basis of retinal-specific induction of cancer by RB mutation

    Cancer Res

    (1999)
  • Cited by (108)

    • Retinoblastoma

      2016, Taylor and Hoyt's Pediatric Ophthalmology and Strabismus, Fifth Edition
    • Ocular Tumors

      2014, Pathobiology of Human Disease: A Dynamic Encyclopedia of Disease Mechanisms
    • Retinoblastoma: present scenario and future challenges

      2023, Cell Communication and Signaling
    View all citing articles on Scopus
    1

    Corresponding author

    View full text