MRPL3

Mitochondrial ribosomal protein L3
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 3J7Y, 3J9M
Identifiers
Symbols	MRPL3 ; COXPD9; MRL3; RPML3
External IDs	OMIM: 607118 HomoloGene: 31431 GeneCards: MRPL3 Gene
Gene ontology Molecular function • RNA binding • structural constituent of ribosome • poly(A) RNA binding Cellular component • mitochondrial inner membrane • mitochondrial large ribosomal subunit Biological process • translation • organelle organization • mitochondrial translation • mitochondrial translational initiation • mitochondrial translational elongation • mitochondrial translational termination Sources: Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	11222	94062
Ensembl	ENSG00000114686	ENSMUSG00000032563
UniProt	P09001	Q99N95
RefSeq (mRNA)	NM_007208	NM_053159
RefSeq (protein)	NP_009139	NP_444389
Location (UCSC)	Chr 3: 131.46 – 131.5 Mb	Chr 9: 105.05 – 105.08 Mb
PubMed search

Mitochondrial ribosomal protein L3 is a protein that in humans is encoded by the MRPL3 gene.^[1]

Mammalian mitochondrial ribosomal proteins are encoded by nuclear genes and help in protein synthesis within the mitochondrion. Mitochondrial ribosomes (mitoribosomes) consist of a small 28S subunit and a large 39S subunit. They have an estimated 75% protein to rRNA composition compared to prokaryotic ribosomes, where this ratio is reversed. Another difference between mammalian mitoribosomes and prokaryotic ribosomes is that the latter contain a 5S rRNA. Among different species, the proteins comprising the mitoribosome differ greatly in sequence, and sometimes in biochemical properties, which prevents easy recognition by sequence homology. This gene encodes a 39S subunit protein that belongs to the L3P ribosomal protein family. A pseudogene corresponding to this gene is found on chromosome 13q. [provided by RefSeq, Jul 2008].^[1]

Clinical relevance

Mutations in this gene have been shown to cause mitochondrial cardiomyopathy.^[2]

References

1. "Entrez Gene: Mitochondrial ribosomal protein L3". Retrieved 2011-12-30.
2. Galmiche L, Serre V, Beinat M, Assouline Z, Lebre AS, Chretien D, Nietschke P, Benes V, Boddaert N, Sidi D, Brunelle F, Rio M, Munnich A, Rötig A (November 2011). "Exome sequencing identifies MRPL3 mutation in mitochondrial cardiomyopathy". Hum. Mutat. 32 (11): 1225–31. doi:10.1002/humu.21562. PMID 21786366.

Further reading

• Kenmochi, N.; Suzuki, T.; Uechi, T.; Magoori, M.; Kuniba, M.; Higa, S.; Watanabe, K.; Tanaka, T. (2001). "The Human Mitochondrial Ribosomal Protein Genes: Mapping of 54 Genes to the Chromosomes and Implications for Human Disorders". Genomics 77 (1–2): 65–70. doi:10.1006/geno.2001.6622. PMID 11543634.
• Ou, J. H.; Yen, T. S.; Wang, Y. F.; Kam, W. K.; Rutter, W. J. (1987). "Cloning and characterization of a human ribosomal protein gene with enhanced expression in fetal and neoplastic cells". Nucleic Acids Research 15 (21): 8919–8934. doi:10.1093/nar/15.21.8919. PMC 306413. PMID 2891103.