GeneID: 445

Description:
The LARP2 gene encodes an RNA-binding protein that belongs to the La-related (LARP) protein family. All family members share the highly conserved La motif but show variability in their RNA recognition structures and, thus, cellular functions. Their mode of action is not clear, but LARPs are proposed to modulate translation elongation or termination and to regulate transcript stability. In fact, the La motif binds to the terminal 3'UUU-OH motif and is the hallmark of RNA polymerase III trancription termination. The genuine La protein provides protection against 3′-exonucleolytic degradation, facilitates correct processing and maturation of RNA polymerase III transcripts, and stimulates translation initiation. Experiments showed that the promoter of the LARP2 gene is highly methylated and its expression is markedly down-regulated in β-thalassaemia patients compared to healthy controls. LARP2 was reported to confer a protective effect on histone deacetylases. The hypermethylation of LARP2 appears to reduce or eliminate HDAC protection, which down-regulates or decreases the production of β-globin. LARP2 plays a role in the regulation of β-globin gene expression and could be a potential modifier of β-thalassaemia severity.

Synonyms: LARP2

Comments:
N/A

Number of entries/variants: 0

1. Bousquet-Antonelli C, Deragon JM, A comprehensive analysis of the La-motif protein superfamily., RNA, 15(5), 750-64, 2009
2. Gao T, Nie Y, Guo J, Hypermethylation of the gene LARP2 for noninvasive prenatal diagnosis of β-thalassemia based on DNA methylation profile., Mol Biol Rep, 39(6), 6591-8, 2012
3. Zakaria NA, Islam MA, Abdullah WZ, Bahar R, Mohamed Yusoff AA, Abdul Wahab R, Shamsuddin S, Johan MF, Epigenetic Insights and Potential Modifiers as Therapeutic Targets in -Thalassemia., Biomolecules, 11(5), 0, 2021