Protein Domain : Leucine-rich PPR motif-containing protein IPR033490

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