CHD2-adjacent suppressive epitranscriptomic regulatory RNA (CHASERR) (CHASERR)

CHASERR (CHD2-adjacent suppressive epitranscriptomic regulatory RNA), also known as LINC01578, is a long non-coding RNA (lncRNA) transcribed from a region immediately upstream of the Chromodomain Helicase DNA Binding Protein 2 (CHD2) gene (Rom et al., 2019, Nature Communications). It functions as a highly conserved cis-regulator that maintains CHD2 homeostasis by suppressing its transcription; loss of CHASERR leads to a significant increase in CHD2 levels (NCBI Gene, 2024). In humans, CHD2 haploinsufficiency is a known cause of severe neurodevelopmental disorders, including Dravet-like syndrome, Lennox-Gastaut syndrome, and autism spectrum disorder (Suls et al., 2013, American Journal of Human Genetics). Because CHASERR naturally limits CHD2 expression, it has emerged as a promising therapeutic target for these conditions. Experimental strategies using antisense oligonucleotides (ASOs) to knock down CHASERR have demonstrated the ability to upregulate the remaining functional CHD2 allele, potentially restoring protein levels to a physiological range (Ionis Pharmaceuticals, 2023). This mechanism offers a potential disease-modifying treatment for patients with CHD2-deficient encephalopathies.

Other names

LINC01578Long intergenic non-protein coding RNA 1578CHD2-AS1CHD2-adjacent suppressive epitranscriptomic regulatory RNA

Knockdown of CHASERR lncRNA using antisense oligonucleotides to relieve the repression of the CHD2 gene, thereby increasing CHD2 protein levels.