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. 2022 May 24;10(6):1229. doi: 10.3390/biomedicines10061229

Figure 2.

Figure 2

Biogenesis of H/ACA box snoRNP. The generation of a H/ACA box snoRNP complex starts with the binding of the SHQ1 protein to dyskerin (DKC1). Subsequently, three additional proteins (nuclear assembly factor 1 (NAF1), H/ACA RNP complex subunit 3/nucleolar protein 10 (NOP10) and H/ACA ribonucleoprotein complex subunit 2 (NHP2)) bind the DKC1-SHQ1 complex, mediated by the RUVBL1-RUVBL2-TAH1-PIH1 (R2TP) complex [20,35]. After the release of SHQ1, this new complex is then transported to the nucleus where it binds the snoRNA sequence on the pre-mRNA. After splicing, the immature H/ACA box snoRNP is translocated to the Cajal bodies where NAF1 is exchanged by H/ACA ribonucleoprotein complex subunit 1 (GAR1). The H/ACA box snoRNP is translocated to the nucleolus by NOPP140, where it can perform the pseudouridylation of target rRNA. Figure modified from reference [35].