SRA stem-loop interacting RNA binding protein (SLIRP)

SLIRP (SRA stem-loop interacting RNA binding protein) is a small RNA-binding protein primarily found in mitochondria and, to a lesser extent, in the nucleus. It forms a complex with LRPPRC, acting as a global RNA chaperone to safeguard mitochondrial mRNA, facilitating their stability, folding, polyadenylation, and efficient translation. In the nucleus, SLIRP binds to specific stem-loop structures within SRA RNA and represses nuclear receptor (NR) transactivation, modulating the effects of coactivators — notably, it can enhance the effect of the drug tamoxifen. Pathogenic variants or depletion of SLIRP have been linked to mitochondrial diseases such as mitochondrial encephalomyopathy and impaired oxidative phosphorylation, highlighting its essential role in maintaining mitochondrial homeostasis and normal cellular energy metabolism. SLIRP's interaction network also connects mitochondrial and nuclear gene regulation, influencing diverse biological processes including cell signaling, apoptosis, and disease pathogenesis.

Other names

SRA stem-loop-interacting RNA-binding protein, mitochondrialSLIRPC14orf156DC23DC50PD04872

Modulation of nuclear receptor activity via SRA RNA interaction. Stabilization and proper folding of mitochondrial transcripts as part of LRPPRC–SLIRP complex. Repression of steroid, androgen, thyroid, and vitamin D receptor-mediated transactivation. Inhibition of mRNA degradation and enhancement of translation in mitochondria.