Target intelligence / Profile preview

Large mitochondrial ribosomal subunit (mt-LSU)

Target
mt-LSU
Molecular classification
Ribosome, Riboprotein complex, Translation machinery, Other
01

Overview

The **large mitochondrial ribosomal subunit** (mt-LSU) is the 39S component of the mammalian mitochondrial ribosome (mitoribosome), an essential machine for synthesizing 13 mitochondrial-encoded proteins critical for oxidative phosphorylation[5][1]. This ribonucleoprotein complex is highly divergent from bacterial and cytoplasmic ribosomes, featuring a drastically reduced rRNA core and an expanded set of proteins, many unique to mitochondria[2][1][7]. The mt-LSU includes the peptidyl transferase center, the nascent polypeptide exit tunnel, and a specialized central protuberance incorporating mitochondrial tRNA^Val^ as a structural component instead of the typical 5S rRNA[1][5][7]. It initiates and catalyzes peptide bond formation during translation of mtDNA-encoded membrane proteins, integrating them into the inner mitochondrial membrane to support electron transport and ATP generation[3][5]. Mutations in mt-LSU components are linked to a variety of human mitochondrial disorders, and many antibiotics can inadvertently inhibit this subunit, resulting in therapeutic challenges and side effects[1][4][9].

Other names
Mitochondrial large ribosomal subunit39S ribosomal subunit (in mammals)Mammalian mitoribosome large subunit
02

Mechanism of action

Inhibition of mitochondrial translation through binding to mitochondrial ribosomal subunits, interfering with the peptidyl transferase center or tRNA sites

03

Biological functions

Mitochondrial protein synthesisTranslation of membrane proteinsOXPHOS complex component biosynthesis
04

Disease associations

Hereditary mitochondrial diseasesOther (disorders of mitochondrial translation or energy metabolism)
05

Safety considerations

Antibiotic toxicity due to off-target effects on human mitochondrial ribosomesMitochondrial dysfunction leading to cytotoxicity and organ-specific toxicity
06

Interacting drugs

Chloramphenicol

3 more in the full profile.

07

Biomarkers

Mutations in mt-LSU proteins or rRNA identified in human mitochondrial disease diagnosticsDefects in OXPHOS complex protein expression

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