RPN1-EVI1 (RPN1-MECOM)
Ribophorin I (RPN1) is a gene that encodes a membrane protein of the rough endoplasmic reticulum that is a component of an N-oligosaccharyl transferase complex (Gene 2013a). In cancer, rearrangements involving RPN1 have been observed in AML.
MDS1 and EVI1 complex locus (MECOM, also known as EVI1) is a gene that encodes a transcriptional regulator protein (Gene 2013b). In cancer, rearrangements involving EVI1 have been observed in AML. Its role is also being investigated in MDS and other cancers.
The RPN1-EVI1 fusion has been observed in AML and CML (Lee et al. 2010).
Contributors: Adam Seegmiller, M.D., Ph.D., Madan Jagasia, M.B.B.S., M.S.
Suggested Citation: Seegmiller, A., M. Jagasia. 2013. RPN1-EVI1 (RPN1-MECOM). My Cancer Genome https://www.mycancergenome.org/content/disease/acute-myeloid-leukemia/rpn1-evi1/?tab=0 (Updated June 4).
Last Updated: June 4, 2013
RPN1-EVI1 (RPN1-MECOM) Fusion Gene in Acute Myeloid Leukemia
Contributors: Adam Seegmiller, M.D., Ph.D., Madan Jagasia, M.B.B.S., M.S., Scott Wheeler, Ph.D. (through June 2014), Cindy L. Vnencak-Jones, Ph.D.
Suggested Citation: Seegmiller, A., M. Jagasia, S. Wheeler, C. Vnencak-Jones. 2013. RPN1-EVI1 (RPN1-MECOM) Fusion Gene in Acute Myeloid Leukemia. My Cancer Genome https://www.mycancergenome.org/content/disease/acute-myeloid-leukemia/rpn1-evi1/ (Updated September 9).
Last Updated: September 9, 2013
RPN1-EVI1 (RPN1-MECOM) t(3;3)(q21;q26.2) Translocation in Acute Myeloid Leukemia
Properties |
Location of translocation |
Chromosomal rearrangements involving the RPN1 gene on 3q21 and the EVI1 gene on 3q26.2 |
Frequency of t(3;3) and inv(3) in AML |
1% (Grimwade et al. 2010; Zhang and Le Beau 2013) |
Implications for Targeted Therapeutics |
Response to FLT3 inhibitors |
Currently no role for RPN1-EVI1 in predicting response |
Response to MEK inhibitors |
Currently no role for RPN1-EVI1 in predicting response |
Response to JAK2 inhibitors |
Currently no role for RPN1-EVI1 in predicting response |
Response to DOT1L inhibitors |
Currently no role for RPN1-EVI1 in predicting response |
The RPN1-EVI1 gene fusion created by the t(3;3)(q21;q26.2) translocation is associated with poor prognosis in AML (NCCN 2012). The official symbol for EVI1 is MECOM.
Contributors: Adam Seegmiller, M.D., Ph.D., Madan Jagasia, M.B.B.S., M.S.
Suggested Citation: Seegmiller, A., M. Jagasia. 2013. RPN1-EVI1 (RPN1-MECOM) t(3;3)(q21;q26.2) Translocation in Acute Myeloid Leukemia. My Cancer Genome https://www.mycancergenome.org/content/disease/acute-myeloid-leukemia/rpn1-evi1/259/ (Updated June 4).
Last Updated: June 4, 2013
My Cancer Genome has released its new and improved cancer clinical trials search tool on our beta website. Please visit beta.mycancergenome.org to check it out!