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

Function: 1) role as transcriptional repressor during development 2) role in control of cell survival 3) overexpression of RERE recruits BAX to the nucleus particularly to POD & triggers caspase-3 activation, leading to cell death 4) interacts with HDAC1 5) interacts with ATN1 via coiled-coil domain - interaction is improved when poly-Gln region of ATN1 is extended Structure: 1) contains 1 BAH domain 2) contains 1 ELM2 domain 3) contains 1 GATA-type zinc finger 4) 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: 1) expressed in tumor cell lines 2) chromosomal translocation t(1;15)(p36.2;q24) involving RERE is found in a neuroblastoma cell line

General

phosphoprotein transcriptional silencer (transcriptional repressor) zinc finger protein

Properties

SIZE: MW = 172 kD entity length = 1566 aa COMPARTMENT: cell nucleus MOTIF: BAH {103-283} ELM2 {284-387} DNA-binding motif SITE: 393-439 Zn finger GATA-type SITE: 507-532 EFFECTOR-BOUND: Zn+2 Ser phosphorylation site {S613} Ser phosphorylation site {S656} proline-rich region SITE: 738-1118 MOTIF: proline residue (SEVERAL) coiled coil {1156-1211} MOTIF: glutamate-rich region {1179-1204} MOTIF: glutamate residue (SEVERAL) glutamate-rich region {1300-1322} MOTIF: glutamate residue (SEVERAL) histidine-rich region {1425-1445} MOTIF: histidine residue (SEVERAL) proline-rich region SITE: 1451-1510 MOTIF: proline residue (SEVERAL)

Database Correlations

OMIM 605226 UniProt Q9P2R6 PFAM correlations

References

UniProt :accession Q9P2R6