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MRPS23

Involvement in disease

Combined oxidative phosphorylation deficiency 46

COXPD46

An autosomal recessive disorder characterized by childhood-onset mitochondrial respiratory chain complex deficiencies, particularly complexes I and IV, and hepatic disease.

None

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

Sequence Similarities

Belongs to the mitochondrion-specific ribosomal protein mS23 family.

Cellular localization

Alternative names

CGI-138, HSPC329, MRPS23, Small ribosomal subunit protein mS23, MRP-S23, S23mt

swissprot:Q9Y3D9 omim:611985 entrezGene:51649