Leucine-rich PPR motif-containing protein (IPR033490)

Short name: LRP130

Overlapping homologous superfamilies

Family relationships



LRP130 is a pentatricopeptide motif protein that binds to mitochondrial and nuclear RNAs [PMID: 12832482]. It associates with mRNA/mRNP complexes at the outside of NE and ER, and plays a role in control of mRNA metabolism [PMID: 15081402]. It may be involved in mitochondrial RNA transcript stability, transcription termination, and RNA processing [PMID: 21971050].

Mutations in the LRP130 gene cause Leigh syndrome French-Canadian type (LSFC), which is a severe neurological disorder characterised by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency [PMID: 12529507].

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.