Target intelligence / Profile preview

Mitochondrial ribosomal protein S25 (MRPS25)

Target
MRPS25
Molecular classification
Ribosomal protein, Mitochondrial small ribosomal subunit protein, Other
01

Overview

Mitochondrial ribosomal protein S25 (MRPS25) is a structural protein component of the 28S small subunit of the mammalian mitochondrial ribosome, essential for mitochondrial protein synthesis. Encoded by a nuclear gene and imported into mitochondria, MRPS25 is one of several proteins that stabilize and assemble the mitoribosome, which translates the 13 essential oxidative phosphorylation (OXPHOS) proteins encoded by mitochondrial DNA. MRPS25 mutations destabilize the small subunit, impairing mitochondrial translation, and cause combined OXPHOS deficiencies clinically manifesting as mitochondrial encephalomyopathy, myopathy, and other neurological deficits. MRPS25 lacks a bacterial homolog and is unique to the mitochondrial ribosome, reflecting evolutionary divergence from prokaryotic ribosomes. Notes: - MRPS25 is not currently considered a therapeutic target such as a receptor, enzyme, or transporter, nor are there known drugs directly targeting it. - There is no evidence for its use as a biomarker or therapeutic agent, but pathogenic mutations can cause rare forms of mitochondrial disease. - The above information uses proper scientific naming and excludes generic, ambiguous, or redundant synonyms.

Other names
Small ribosomal subunit protein mS25MRPS25RPMS25MRP-S25S25mtmS25COXPD5028S ribosomal protein S25 (mitochondrial)mitochondrial 28S ribosomal protein S25mitochondrial small ribosomal subunit protein mS25FLJ00023DKFZp313H0817
02

Biological functions

Mitochondrial protein synthesisAssembly and stability of mitochondrial ribosome small subunit (28S)Maintenance of mitochondrial translation and respiratory chain function
03

Disease associations

Mitochondrial encephalomyopathyMitochondrial myopathyBrain abnormalities (including cerebral palsy, partial agenesis of corpus callosum)Combined oxidative phosphorylation (OXPHOS) deficiency
04

Safety considerations

MRPS25 gain-of-function in normal settings may be toxic to cells, as lentivirally overexpressed MRPS25 caused cell death in wild-type fibroblasts

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