Mitochondrial assembly of ribosomal large subunit protein 1 (MALSU1)

Mitochondrial assembly of ribosomal large subunit protein 1 (MALSU1) is a critical assembly factor that functions during the late stages of mitochondrial ribosome (mitoribosome) biogenesis[1][2]. MALSU1 is part of a regulatory module (with L0R8F8 and mtACP) that transiently binds the mitochondrial large ribosomal subunit to prevent premature subunit joining, ensuring that only fully matured 55S monosomes participate in protein synthesis[1][3][4]. By interacting with mitoribosomal proteins such as uL14m, MALSU1 contributes to the quality control checkpoint of mitochondrial translation, which is essential for efficient oxidative phosphorylation. MALSU1 belongs to the ribosome silencing factor family, and its activity is required to regulate the maturation and assembly of the mitochondrial large ribosomal subunit[3]. Depletion of MALSU1 results in defective mitochondrial ribosome assembly and impaired mitochondrial protein synthesis[1][2][3]. There are currently no therapies that specifically target MALSU1. Defects or mutations in MALSU1 have been linked to rare mitochondrial syndromes but not to common disease phenotypes[2].

Other names

C7orf30mtRsfAMTRSFAMASU1_HUMAN

Not applicable; no drugs known to target this assembly factor