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Free keywords:
Animals
Conserved Sequence
HeLa Cells
Humans
Mice
Mice, Inbred C57BL
Mitochondria/*metabolism
Mitochondrial Proteins/classification/genetics/*metabolism
Neoplasm Proteins/classification/genetics/*metabolism
Phylogeny
Protein Structure, Tertiary
Protein Transport
Repetitive Sequences, Amino Acid
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.
