LRPPRC is a mitochondrial matrix protein that is conserved in metazoans.
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.
Sterky
Fredrik H
FH
Department of Laboratory Medicine, Karolinska Institutet, Retzius väg 8, SE-171 77 Stockholm, Sweden.
Ruzzenente
Benedetta
B
Gustafsson
Claes M
CM
Samuelsson
Tore
T
Larsson
Nils-Göran
NG
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
C087940:LRPPRC protein, human; D024101:Mitochondrial Proteins; D009363:Neoplasm Proteins