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MRPS25

Involvement in disease

Combined oxidative phosphorylation deficiency 50

COXPD50

An autosomal recessive, mitochondrial encephalomyopathy characterized by intrauterine growth retardation, poor overall growth, delayed psychomotor development, hypotonia, muscle weakness, progressive loss of ambulation, and mitochondrial oxidative phosphorylation deficiency in patient tissues. Brain imaging shows partial agenesis of the corpus callosum.

None

The disease may be caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the mitochondrion-specific ribosomal protein mS25 family.

Cellular localization

Alternative names

RPMS25, MRPS25, Small ribosomal subunit protein mS25, MRP-S25, S25mt

swissprot:P82663 entrezGene:64432 omim:611987