LRPPRC
LRPPRC (leucine-rich pentatricopeptide repeat containing, also known as GP130, LRP130 and LSFC) is a mitochondrial protein that binds RNA and supports the expression of mitochondrially encoded genes. It also acts in the nucleus, where it is linked to mRNA export and transcription regulation. Browse Assay Genie's current range of LRPPRC research reagents below, or read on for the biology behind the target.
LRPPRC is thought to play a role in RNA metabolism in both mitochondria and the nucleus. In mitochondria it binds poly(A) mRNA and contributes to the translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. It is also required for maintaining mitochondrial potential, and it suppresses the initiation of basal autophagy and mitophagy by sustaining BCL2 levels.
In the nucleus, LRPPRC binds HNRPA1-associated poly(A) mRNAs and is part of nmRNP complexes at late stages of mRNA maturation, possibly associated with nuclear mRNA export. It positively modulates export of mRNAs carrying the EIF4E sensitivity element (4ESE) by binding EIF4E and the 4ESE at the same time, acting as a platform for assembly of the export complex. It also binds exportin XPO1/CRM1 to engage the nuclear pore and traffic bound mRNAs to the cytoplasm. LRPPRC may additionally be involved in transcription regulation: it cooperates with PPARGC1A to regulate certain mitochondrially encoded and gluconeogenic genes, and it is part of a nuclear factor that binds the invMED1 element of the MDR1 and MVP gene promoters. It also binds single-stranded DNA.
The LRPPRC gene encodes a leucine-rich protein with multiple pentatricopeptide repeats (PPR). The protein localises primarily to mitochondria and is predicted to carry an N-terminal mitochondrial targeting sequence. HGNC places it among mitochondrial respiratory chain complex assembly factors, mitochondrial nucleoid-associated proteins and the LRPPRC:SLIRP complex subunits. Mutations in the gene are associated with the French-Canadian type of Leigh syndrome, which explains the former gene name and the LSFC alias. Studies also suggest roles in cytoskeletal organisation and vesicular transport, although the precise role remains unclear.
LRPPRC influences the translation or stability of mitochondrially encoded COX subunits, making it a focus for studies of mitochondrial RNA handling.
Mutations in the LRPPRC gene are associated with French-Canadian type Leigh syndrome, linking the protein to cytochrome oxidase deficiency.
LRPPRC assembles an export complex on 4ESE-containing mRNAs with EIF4E and XPO1/CRM1, supporting work on selective mRNA trafficking.
By sustaining BCL2 levels, LRPPRC suppresses basal autophagy and mitophagy, and it helps maintain mitochondrial potential.
LRPPRC is thought to function in RNA metabolism in mitochondria and the nucleus. In mitochondria it binds poly(A) mRNA and supports COX subunit translation or stability. In the nucleus it aids mRNA export and may regulate transcription. It also helps maintain mitochondrial potential.
The HGNC full name is leucine rich pentatricopeptide repeat containing, and UniProt lists it as leucine-rich PPR motif-containing protein, mitochondrial. Aliases include GP130, LRP130 and LSFC. LSFC derives from the earlier name Leigh syndrome, French-Canadian type (cytochrome oxidase deficiency).
Mouse and rat both have an ortholog named Lrpprc, so the protein can be studied in common rodent models. In humans, mutations in the gene are associated with the French-Canadian type of Leigh syndrome. Check the datasheet of any product for its validated reactivity.
Mitochondrial RNA-processing factors, nuclear export proteins and transcription regulators connected to LRPPRC biology.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P42704; product specifications from Assay Genie datasheets.