title: LRPPRC is a mitochondrial matrix protein that is conserved in metazoans.
abstract: LRPPRC (also called LRP130) is an RNA-binding pentatricopeptide repeat protein. LRPPRC has been recognized as a mitochondrial protein, but has also been shown to regulate nuclear gene transcription and to bind specific RNA molecules in both the nucleus and the cytoplasm. We here present a bioinformatic analysis of the LRPPRC primary sequence, which reveals that orthologs to the LRPPRC gene are restricted to metazoan cells and that all of the corresponding proteins contain mitochondrial targeting signals. To address the subcellular localization further, we have carefully analyzed LRPPRC in mammalian cells and identified a single isoform that is exclusively localized to mitochondria. The LRPPRC protein is imported to the mitochondrial matrix and its mitochondrial targeting sequence is cleaved upon entry.
authors.affiliation: Department of Laboratory Medicine, Karolinska Institutet, Retzius väg 8, SE-171 77 Stockholm, Sweden.
authors.forename: Fredrik H
authors.initials: FH
authors.lastname: Sterky
authors.forename: Benedetta
authors.initials: B
authors.lastname: Ruzzenente
authors.forename: Claes M
authors.initials: CM
authors.lastname: Gustafsson
authors.forename: Tore
authors.initials: T
authors.lastname: Samuelsson
authors.forename: Nils-Göran
authors.initials: NG
authors.lastname: Larsson
mesh_terms: D000818:Animals; D017124:Conserved Sequence; D006367:HeLa Cells; D006801:Humans; D051379:Mice; D008810:Mice, Inbred C57BL; D008928:Mitochondria / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000145:classification / Q000235:genetics / Q000378:metabolism*; D009363:Neoplasm Proteins / Q000145:classification / Q000235:genetics / Q000378:metabolism*; D010802:Phylogeny; D017434:Protein Structure, Tertiary; D021381:Protein Transport; D020449:Repetitive Sequences, Amino Acid
chemical_list: C087940:LRPPRC protein, human; D024101:Mitochondrial Proteins; D009363:Neoplasm Proteins