| Primary Identifier | IPR031271 | Type | Family |
| Short Name | RCAN1 |
| description | Calcipressin-1 (RCAN1, also known as DSCR1) is a calcineurin inhibitor that inactivates nuclear factor of activated T cells (NFATc) activity []. It is expressed in the primitive ventricle of the heart and during neurogenesis []. It is a phosphoprotein that increases its capacity to inhibit calcineurin when phosphorylated at the FLISPP motif, and this phosphorylation also controls the half-life of calcipressin 1 by accelerating its degradation []. The DSCR1 (Down syndrome critical region 1) gene is present in the region of human chromosome 21, trisomy of which is associated with congenital heart defects observed in Down syndrome [].Calcipressins (RCANs) are a novel family of calcineurin regulators that have been suggested as key factors contributing to Down syndrome in humans. Three human calcipressins have been identified, calcipressin 1-3. Calcipressin 1 is also known as modulatory calcineurin-interacting protein 1 (MCIP1), Adapt78 and Down syndrome critical region 1 (DSCR1). Calcipressin 2 is variously known as MCIP2, ZAKI-4 and DSCR1-like 1. Calcipressin 3 is also called MCIP3 and DSCR1-like 2 []. Calcipressins contain an N-terminal RNA recognition motif (RRM), a highly conserved SP repeat domain containing the phosphorylation site by GSK-3, a well-known PxIxIT motif responsible for docking many substrates to calcineurin, and a C-terminal TxxP motif [].Calcineurin is a calcium-responsive enzyme that dephosphorylates the nuclear factor of activated T cells (NFAT). In doing so, it promotes its nuclear translocation and uniquely links calcium signalling to transcriptional regulation []. |
