26lk
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Cryo-EM structure of human LAS1L-NOL9 complex
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Structural highlights
DiseaseLAS1L_HUMAN Spinal muscular atrophy with respiratory distress type 2;Wilson-Turner syndrome. The disease is caused by variants affecting the gene represented in this entry. FunctionLAS1L_HUMAN Required for the synthesis of the 60S ribosomal subunit and maturation of the 28S rRNA (PubMed:20647540). Functions as a component of the Five Friends of Methylated CHTOP (5FMC) complex; the 5FMC complex is recruited to ZNF148 by methylated CHTOP, leading to desumoylation of ZNF148 and subsequent transactivation of ZNF148 target genes (PubMed:22872859). Required for the efficient pre-rRNA processing at both ends of internal transcribed spacer 2 (ITS2) (PubMed:22083961).[1] [2] [3] References
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This page was last modified 06:41, 3 June 2026.