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1.

Retinoblastoma

A malignant tumor arising from the nuclear layer of the retina that is the most common primary tumor of the eye in children. The tumor tends to occur in early childhood or infancy and may be present at birth. The majority are sporadic, but the condition may be transmitted as an autosomal dominant trait. Histologic features include dense cellularity, small round polygonal cells, and areas of calcification and necrosis. An abnormal pupil reflex (leukokoria); NYSTAGMUS, PATHOLOGIC; STRABISMUS; and visual loss represent common clinical characteristics of this condition. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2104)

2.

Retinoblastoma-Binding Protein 7

A retinoblastoma-binding protein that has an affinity for core HISTONES. It is found as a subunit of protein complexes that are in involved in the enzymatic modification of histones including the Mi2 and Sin3 histone deacetylase complexes and the polycomb repressive complex 2.

Year introduced: 2010

3.

Retinoblastoma-Binding Protein 4

A retinoblastoma-binding protein that is involved in CHROMATIN REMODELING, histone deacetylation, and repression of GENETIC TRANSCRIPTION. Although initially discovered as a retinoblastoma binding protein it has an affinity for core HISTONES and is a subunit of chromatin assembly factor-1 and polycomb repressive complex 2.

Year introduced: 2010

4.

Retinoblastoma-Binding Protein 2

A retinoblastoma binding protein that is also a member of the Jumonji-domain histone demethylases. It has demethylation activity towards specific LYSINE residues found on HISTONE H3.

Year introduced: 2010

5.

Retinoblastoma-Binding Protein 1

A ubiquitously expressed regulatory protein that contains a retinoblastoma protein binding domain and an AT-rich interactive domain. The protein may play a role in recruiting HISTONE DEACETYLASES to the site of RETINOBLASTOMA PROTEIN-containing transcriptional repressor complexes.

Year introduced: 2010

6.

Retinoblastoma Binding Proteins

A family of endogenous regulatory proteins that associate with RETINOBLASTOMA PROTEIN via a specific high-affinity binding domain. Members of this family of proteins are often found associated with histone-modifying enzymes and protein complexes that regulate gene expression.

Year introduced: 2010

7.

Retinoblastoma-Like Protein p107

A negative regulator of the CELL CYCLE that undergoes PHOSPHORYLATION by CYCLIN-DEPENDENT KINASES. It contains a conserved pocket region that binds E2F4 TRANSCRIPTION FACTOR and interacts with viral ONCOPROTEINS such as POLYOMAVIRUS TUMOR ANTIGENS; ADENOVIRUS E1A PROTEINS; and PAPILLOMAVIRUS E7 PROTEINS.

Year introduced: 2006(1991)

8.

Retinoblastoma-Like Protein p130

A negative regulator of the CELL CYCLE that undergoes PHOSPHORYLATION by CYCLIN-DEPENDENT KINASES. RBL2 contains a conserved pocket region that binds E2F4 TRANSCRIPTION FACTOR and E2F5 TRANSCRIPTION FACTOR. RBL2 also interacts with viral ONCOPROTEINS such as POLYOMAVIRUS TUMOR ANTIGENS; ADENOVIRUS E1A PROTEINS; and PAPILLOMAVIRUS E7 PROTEINS.

Year introduced: 2006(1994)

9.

Genes, Retinoblastoma

Tumor suppressor genes located on human chromosome 13 in the region 13q14 and coding for a family of phosphoproteins with molecular weights ranging from 104 kDa to 115 kDa. One copy of the wild-type Rb gene is necessary for normal retinal development. Loss or inactivation of both alleles at this locus results in retinoblastoma.

Year introduced: 1991

10.

Retinoblastoma Protein

Product of the retinoblastoma tumor suppressor gene. It is a nuclear phosphoprotein hypothesized to normally act as an inhibitor of cell proliferation. Rb protein is absent in retinoblastoma cell lines. It also has been shown to form complexes with the adenovirus E1A protein, the SV40 T antigen, and the human papilloma virus E7 protein.

Year introduced: 1991

11.

E2F1 Transcription Factor

An E2F transcription factor that interacts directly with RETINOBLASTOMA PROTEIN and CYCLIN A and activates GENETIC TRANSCRIPTION required for CELL CYCLE entry and DNA synthesis. E2F1 is involved in DNA REPAIR and APOPTOSIS.

Year introduced: 2006(1991)

12.

RBL1 protein, human [Supplementary Concept]

RefSeq NM_002895

Date introduced: July 29, 2005

13.

RBL2 protein, human [Supplementary Concept]

RefSeq NM_005611

Date introduced: July 29, 2005

14.

RBBP4 protein, human [Supplementary Concept]

RefSeq NM_005610

Date introduced: September 13, 1993

15.

Rbl2 protein, rat [Supplementary Concept]

RefSeq NM_031094

Date introduced: July 29, 2005

16.

E2F1 protein, human [Supplementary Concept]

RefSeq NM_005225

Date introduced: July 29, 2005

17.

Rbbp4 protein, mouse [Supplementary Concept]

RefSeq NM_009030

Date introduced: September 29, 2004

18.

Rbl1 protein, mouse [Supplementary Concept]

cellular protein that forms specific complexes with adenovirus E1A & SV40 large T antigen; inhibits E2F-mediated transactivation; RefSeq NM_011249

Date introduced: September 29, 2004

19.

RBBP9 protein, human [Supplementary Concept]

RefSeq NM_006606

Date introduced: November 2, 2004

20.

Rbbp7 protein, mouse [Supplementary Concept]

RefSeq NM_009031

Date introduced: November 30, 1995

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