arginine-glutamic acid dipeptide repeats protein (atrophin-1-like protein, atrophin-1-related protein, RERE, ARG, ARP, ATN1L, KIAA0458)

Function

• role as transcriptional repressor during development
• role in control of cell survival
• overexpression of RERE recruits BAX to the nucleus particularly to POD & triggers caspase-3 activation, leading to cell death
• interacts with HDAC1
• interacts with ATN1 via coiled-coil domain
  • interaction is improved when poly-Gln region of ATN1 is extended

Structure

• contains 1 BAH domain
• contains 1 ELM2 domain
• contains 1 GATA-type zinc finger
• contains 1 Myb DNA-binding domain

Compartment

• nucleus, localized in nuclear bodies
• colocalized with PML & BAX in nuclear PODs

Alternative splicing

named isoforms=2

Expression

• widely expressed

Pathology

• expressed in tumor cell lines
• chromosomal translocation t(1;15)(p36.2;q24) involving RERE is found in a neuroblastoma cell line

More general terms

• transcriptional silencer (transcriptional repressor)
• zinc finger protein
• phosphoprotein

Database

• OMIM: https://mirror.omim.org/entry/605226
• UniProt: http://www.uniprot.org/uniprot/Q9P2R6.html