UniProt ID: Q6PEH4 |
FUNCTION: Component of the ribosomal small subunit processome for the biogenesis of ribosomes, functions in pre-ribosomal RNA (pre-rRNA) processing (PubMed:28263972). Essential for embryonic development in part through the regulation of p53 pathway. Required for the expansion growth of intestine, liver and exocrine pancreas, but not endocrine pancreas. May not play a major role during the early stage of endodermal organogenesis. Also involved in the sympathetic neuronal development (PubMed:28263972). Triggers the degradation of p53/TP53 and Delta113p53 isoform in a ubiquitination pathway independent way (PubMed:23357851). Mediates, with CAPN3, the proteasome-independent degradation of p53/TP53 (PubMed:23357851, PubMed:27657329). {ECO:0000269|PubMed:12006978, ECO:0000269|PubMed:16322560, ECO:0000269|PubMed:23357851, ECO:0000269|PubMed:27657329, ECO:0000269|PubMed:28263972}. SUBCELLULAR LOCATION: Nucleus, nucleolus {ECO:0000269|PubMed:16322560, ECO:0000269|PubMed:23357851}. TISSUE SPECIFICITY: Mainly expressed in digestive organs at the later stage of organogenesis. Expressed in liver, gut and exocrine pancreas, but not in the islets. {ECO:0000269|PubMed:16322560, ECO:0000269|PubMed:23357851}. DEVELOPMENTAL STAGE: Highly expressed in the embryos at 12 hpf and 1 dpf and expressed at high level at 3 dpf and 4 dpf, but at a lower level at 5 dpf. At 2 dpf, ubiquitously expressed, but enriched in the mid and hindbrain boundary. Through 3-5 dpf, expressed mainly in the gut, liver, pancreas and pharynx. {ECO:0000269|PubMed:16322560}. PTM: Phosphorylated. Phosphorylation is required to facilitate the nucleolar localzsation of CAPN3 and promote p53/TP53 degradation in the nucleolus which promotes cell cycle progression and liver development. {ECO:0000269|PubMed:27657329}. DISRUPTION PHENOTYPE: Confers hypoplastic digestive organs and selectively up-regulates the expression of the Delta113p53 isoform but not p53, resulting in compromised organ growth in def mutant fish. Loss of function affects sympathetic neuronal development (PubMed:27657329). {ECO:0000269|PubMed:16322560, ECO:0000269|PubMed:23357851, ECO:0000269|PubMed:27657329}. SIMILARITY: Belongs to the UTP25 family. {ECO:0000305}. |
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