Leucine-rich PPR motif-containing protein, mitochondrial (LRPPRC)

Leucine-rich PPR motif-containing protein, mitochondrial (LRPPRC) is a multifunctional RNA-binding protein that plays a fundamental role in mitochondrial gene expression by stabilizing and processing mitochondrial mRNA and modulating its translation[1][2][3][4]. It contains tandem pentatricopeptide repeats, enabling it to bind, stabilize, and chaperone mitochondrial mRNAs, particularly as a complex with the cofactor SLIRP[3]. LRPPRC is crucial for polyadenylation and efficient translation of several mitochondrial transcripts, thus controlling energy metabolism in tissues highly dependent on mitochondrial respiration. Mutations in LRPPRC cause Leigh syndrome, French-Canadian type (LSFC), a severe pediatric neurodegenerative disorder due to impaired mitochondrial energy conversion[1][3]. Dysregulation (especially overexpression) of LRPPRC is linked to tumor progression and poor prognosis in several cancers, and the protein has emerging associations with other diseases such as Parkinson's disease and viral infections. LRPPRC operates at multiple cellular locations, ranging from the mitochondria to the nucleoplasm and endoplasmic reticulum, and also interacts with diverse cellular complexes involved in RNA metabolism and vesicular trafficking[1][2][3][4]. Despite its pathogenic importance, LRPPRC is not yet a direct therapeutic drug target, but its critical cellular functions make it an attractive molecule for research into disease mechanisms and biomarker development.

Other names

LRPPRCLRP130GP130130 kDa leucine-rich proteinLSFCMC4DN5CLONE-23970