The Biology of Retinoblastoma.
Prog Mol Biol Transl Sci
; 134: 503-16, 2015.
Article
in En
| MEDLINE
| ID: mdl-26310174
ABSTRACT
Retinoblastoma, the most common primary intraocular cancer of childhood, is a malignancy arising in the developing retina. Tumor formation usually begins with mutation in both alleles of the retinoblastoma tumor suppressor gene RB1, followed by a series of other genetic alterations that correlate with the clinical stage and pathologic findings of the tumor. Analysis of sporadic and heritable retinoblastoma led to the development of Knudson's Two-Hit Hypothesis. The tumor suppressor RB1 gene codes for the retinoblastoma protein which is a key regulator of cellular replication via its binding to the E2F family of transcription factors and chromatin remodeling proteins. Studies of preclinical models of retinoblastoma in the form of transgenic mice and xenograft animal models have significantly contributed to the development of effective therapies for this disease. Research on retinoblastoma has paved the way toward understanding many of the mechanisms in cancer genetics.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Retinoblastoma
Type of study:
Prognostic_studies
Limits:
Animals
/
Humans
Language:
En
Journal:
Prog Mol Biol Transl Sci
Journal subject:
BIOLOGIA MOLECULAR
Year:
2015
Document type:
Article
Affiliation country:
United States