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Leucine-rich pentatricopeptide repeat-containing protein, mitochondrial (LRPPRC)
- Target
- LRPPRC
- Molecular classification
- Other (RNA-binding protein, mitochondrial protein)
01
Overview
Leucine-rich pentatricopeptide repeat-containing protein, mitochondrial (LRPPRC), is a human protein encoded by the LRPPRC gene. The protein is characterized by multiple pentatricopeptide repeat (PPR) motifs and is leucine-rich. It primarily plays roles in the regulation of mitochondrial gene expression by binding mitochondrial RNAs, particularly affecting the expression of cytochrome c oxidase subunits (MT-CO1 and MT-CO3). Mutations in LRPPRC cause the French-Canadian variant of Leigh syndrome, a severe neurodegenerative disorder, and it has been implicated in the progression and immune evasion of certain cancers, such as hepatocellular carcinoma, via modulation of m(6)A modification of PD-L1 mRNA.
- Other names
- GP130LRP130LSFCCLONE-23970leucine rich pentatricopeptide repeat containingMC4DN5
02
Biological functions
Regulation of cytoskeleton interactionmitochondrial gene expression regulationRNA bindingregulation of MT-CO1 and MT-CO3 expression
03
Disease associations
Neurodegenerative disease (Leigh syndrome, French-Canadian type)Cancer (tumor progression in hepatocellular carcinoma)
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