Era like 12S mitochondrial rRNA chaperone 1 (ERAL1)

Era like 12S mitochondrial rRNA chaperone 1 (ERAL1) is a mitochondrial GTPase that acts as an essential RNA chaperone during the biogenesis of the mitochondrial small ribosomal subunit (28S). ERAL1 binds specifically to a 33-nucleotide region at the 3′ terminal stem-loop of the mitochondrial 12S ribosomal RNA, stabilizing it during ribosome assembly. Loss or depletion of ERAL1 disrupts proper mitoribosome formation, leading to instability and decay of 12S mt-rRNA, cell growth defects, and apoptosis, but does not immediately disrupt mitochondrial protein synthesis. Mutations or deficiency in ERAL1 are associated with Perrault syndrome 6 and contribute to mitochondrial dysfunction and respiratory chain deficiencies[1][2][3][4]. ERAL1’s primary role is non-structural, acting as a chaperone and protector for 12S mt-rRNA during ribosome assembly and ensuring mitochondrial gene expression and cell survival.

Other names

GTPase Era, mitochondrialHERAH-ERAhERACEGAHERA-BConserved ERA-like GTPaseERA-WERA-like protein 1ERAL1AERAL1BHERA-APRLTS6GTP-binding protein era homologGTPase, human homolog of E. coli essential cell cycle protein Eraera (E. coli G-protein homolog)-like 1