Reactome | NCOA1(868-1441) binds PAX3(1-319)

NCOA1(868-1441) binds PAX3(1-319)

Stable Identifier

R-HSA-5579018

Type

Reaction [binding]

Species

Homo sapiens

Compartment

nucleoplasm

Synonyms

NCOA1:PAX3 oncogene causes rhabdomyosarcoma

ReviewStatus

5/5

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A chromosomal aberration involving the nuclear receptor coactivator NCOA1 and paired box protein Pax-3 (PAX3) is a cause of rhabdomyosarcoma (RMS). Translocation t(2;2)(q35;p23) with PAX3 generates the NCOA1-PAX3 oncogene consisting of the N-terminus part of PAX3 and the C-terminus part of NCOA1. The fusion protein acts as a transcriptional activator. RMS is the most common soft tissue carcinoma in childhood, representing 5-8% of all malignancies in children (Wachtel et al. 2004).

Literature References

PubMed ID	Title	Journal	Year
15313887	Gene expression signatures identify rhabdomyosarcoma subtypes and detect a novel t(2;2)(q35;p23) translocation fusing PAX3 to NCOA1 Schäfer, BW, Stegmaier, S, Simon-Klingenstein, K, Dettling, M, Wachtel, M, Koscielniak, E, Treuner, J, Niggli, FK, Bühlmann, P	Cancer Res.	2004