Mitochondrial ribosomal protein S2 (MRPS2)

Mitochondrial ribosomal protein S2 (MRPS2) is a nucleus-encoded protein that forms a critical part of the small (28S) subunit of the mitochondrial ribosome, which is responsible for synthesizing the thirteen proteins encoded by mitochondrial DNA and essential for the assembly and function of the oxidative phosphorylation (OXPHOS) system[1][2][3]. Disruption of MRPS2 impairs assembly of the small mitoribosomal subunit, leading to compromised mitochondrial translation, inefficiency of ATP generation, and reduced cellular energy production. Pathogenic variants in MRPS2 are associated with a combined OXPHOS deficiency syndrome (OMIM #611971), which can manifest as lactic acidemia, hypoglycemia, and sensorineural hearing loss[1][2]. MRPS2 is not traditionally considered a direct drug target (receptor, enzyme, transporter or similar therapeutic target class)[1][2][3].

Other names

Small ribosomal subunit protein uS2mMRP-S2S2mtCGI-91uS2m28S ribosomal protein S2, mitochondrialCOXPD36