Little elongation complex subunit 1 (ICE1)

Little elongation complex subunit 1 (ICE1) is a large (~250 kDa) nuclear protein that functions as a critical scaffold component of the little elongation complex (LEC), a multi-protein complex essential for the regulation of small nuclear RNA (snRNA) gene transcription by RNA polymerase II and III[1][2][3][4]. ICE1 enables the assembly and stability of the LEC, thereby contributing to the accurate elongation of snRNA transcripts and facilitating proper recruitment of RNA polymerase II at snRNA gene promoters[1][5][7]. Beyond its role in snRNA biogenesis, ICE1 also participates in RNA surveillance mechanisms, specifically by promoting the link between splicing and nonsense-mediated mRNA decay (NMD) through a conserved C-terminal MIF4G domain[2][3][4]. ICE1 interacts transiently with the exon junction complex (EJC), and its depletion disrupts the association between the NMD factor UPF3B and the EJC, impairing NMD efficiency[2][3][4]. Diseases associated with ICE1 include Cri-du-chat syndrome and Tinea nigra, and its locus in human genetic studies implicates it in neurodevelopment via snRNA processing[1][3]. ICE1 does not have well-characterized direct interactions with drugs, nor is it a typical therapeutic target such as a receptor, enzyme, or transporter[1][3][5]. Overall, ICE1 is a context-dependent, evolutionarily conserved regulator of non-coding RNA transcription and RNA metabolism, acting primarily as a structural and regulatory scaffold in its complexes rather than as an enzyme or classical drug target[1][3][4][5][7].

Other names

Interactor of little elongation complex ELL subunit 1KIAA0947ICE1Interactor of little elongator complex ELL subunit 1