{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nSNRPB is a core spliceosomal component that is essential for the regulation of pre‐mRNA splicing and proper developmental programs. A series of studies have shown that its own pre‐mRNA undergoes alternative splicing regulated by a conserved intragenic exon; mutations that enhance inclusion of this premature termination codon–harboring exon lead to aberrant SNRPB expression and are the genetic basis of cerebro‐costo‐mandibular syndrome (CCMS). In addition, comparative genomic analyses suggest that the ancestral SNRPB gene evolved distinct splice variants (e.g., SmB and SmB′) and is subject to dosage compensation mechanisms that maintain the proper stoichiometry of spliceosomal components."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "1", "end_ref": "4"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nAberrant SNRPB expression has been implicated in multiple cancer types, where its dysregulation contributes to oncogenesis through mis‐regulated alternative splicing. In glioblastoma, as part of broader studies on RNA‑binding proteins, SNRPB is highlighted as a potential contributor to tumor biology, while a single nucleotide polymorphism in SNRPB has been associated with modified breast cancer risk in BRCA1 mutation carriers. In non‑small cell lung carcinoma, overexpression of SNRPB promotes tumorigenesis by altering splicing of key transcripts such as RAB26 via intron retention and nonsense‑mediated decay. Elevated levels of SNRPB in hepatocellular and liver cancers have been linked to enhanced cell proliferation, stemness, epithelial‑to‑mesenchymal transition, and metabolic reprogramming via the Akt signaling pathway. Its oncogenic role is further supported in cervical and ovarian cancers, where SNRPB interacts with p53 and modulates splicing events that affect DNA replication and homologous recombination."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "5", "end_ref": "13"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nApart from its roles in development and tumorigenesis, SNRPB is pivotal in the assembly and biogenesis of spliceosomal snRNPs. Evidence indicates that its incorporation into snRNP complexes is tightly regulated by post‑translational modifications and interactions with factors such as coilin and the survival of motor neuron (SMN) protein, which coordinate snRNP trafficking and maturation. Moreover, alterations in arginine methylation have been demonstrated to affect SNRPB binding and splicing kinetics, underscoring its critical function in efficient post‑transcriptional RNA processing and overall RNA metabolism."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "14", "end_ref": "16"}]}, {"type": "t", "text": "\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Danielle C Lynch, Timothée Revil, Jeremy Schwartzentruber, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Disrupted auto-regulation of the spliceosomal gene SNRPB causes cerebro-costo-mandibular syndrome."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Nat Commun (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/ncomms5483"}], "href": "https://doi.org/10.1038/ncomms5483"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25047197"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25047197"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Séverine Bacrot, Mathilde Doyard, Céline Huber, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Mutations in SNRPB, encoding components of the core splicing machinery, cause cerebro-costo-mandibular syndrome."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hum Mutat (2015)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/humu.22729"}], "href": "https://doi.org/10.1002/humu.22729"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25504470"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25504470"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Madeleine Tooley, Danielle Lynch, Francois Bernier, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Cerebro-costo-mandibular syndrome: Clinical, radiological, and genetic findings."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Am J Med Genet A (2016)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/ajmg.a.37587"}], "href": "https://doi.org/10.1002/ajmg.a.37587"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "26971886"}], "href": "https://pubmed.ncbi.nlm.nih.gov/26971886"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "T A Gray, M J Smithwick, M A Schaldach, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Concerted regulation and molecular evolution of the duplicated SNRPB'/B and SNRPN loci."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Nucleic Acids Res (1999)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1093/nar/27.23.4577"}], "href": "https://doi.org/10.1093/nar/27.23.4577"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "10556313"}], "href": "https://pubmed.ncbi.nlm.nih.gov/10556313"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Bruna R Correa, Patricia Rosa de Araujo, Mei Qiao, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Functional genomics analyses of RNA-binding proteins reveal the splicing regulator SNRPB as an oncogenic candidate in glioblastoma."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Genome Biol (2016)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1186/s13059-016-0990-4"}], "href": "https://doi.org/10.1186/s13059-016-0990-4"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "27287018"}], "href": "https://pubmed.ncbi.nlm.nih.gov/27287018"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Xianshu Wang, V Shane Pankratz, Zachary Fredericksen, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Common variants associated with breast cancer in genome-wide association studies are modifiers of breast cancer risk in BRCA1 and BRCA2 mutation carriers."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hum Mol Genet (2010)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1093/hmg/ddq174"}], "href": "https://doi.org/10.1093/hmg/ddq174"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "20418484"}], "href": "https://pubmed.ncbi.nlm.nih.gov/20418484"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Nianli Liu, Zhiyuan Wu, Aoxing Chen, et al. "}, {"type": "b", "children": [{"type": "t", "text": "SNRPB promotes the tumorigenic potential of NSCLC in part by regulating RAB26."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell Death Dis (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/s41419-019-1929-y"}], "href": "https://doi.org/10.1038/s41419-019-1929-y"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31511502"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31511502"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Yu-Ting Zhan, Lei Li, Ting-Ting Zeng, et al. "}, {"type": "b", "children": [{"type": "t", "text": "SNRPB-mediated RNA splicing drives tumor cell proliferation and stemness in hepatocellular carcinoma."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Aging (Albany NY) (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.18632/aging.202164"}], "href": "https://doi.org/10.18632/aging.202164"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "33289700"}], "href": "https://pubmed.ncbi.nlm.nih.gov/33289700"}]}, {"type": "r", "ref": 9, "children": [{"type": "t", "text": "Lei Zhu, Xiuzhen Zhang, Ziqin Sun "}, {"type": "b", "children": [{"type": "t", "text": "SNRPB promotes cervical cancer progression through repressing p53 expression."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biomed Pharmacother (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.biopha.2020.109948"}], "href": "https://doi.org/10.1016/j.biopha.2020.109948"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "32106364"}], "href": "https://pubmed.ncbi.nlm.nih.gov/32106364"}]}, {"type": "r", "ref": 10, "children": [{"type": "t", "text": "Yingwei Li, Zhongshao Chen, Jiali Peng, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The splicing factor SNRPB promotes ovarian cancer progression through regulating aberrant exon skipping of POLA1 and BRCA2."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Oncogene (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/s41388-023-02763-x"}], "href": "https://doi.org/10.1038/s41388-023-02763-x"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "37391593"}], "href": "https://pubmed.ncbi.nlm.nih.gov/37391593"}]}, {"type": "r", "ref": 11, "children": [{"type": "t", "text": "Ningfu Peng, Jindu Li, Jingrong He, et al. "}, {"type": "b", "children": [{"type": "t", "text": "c-Myc-mediated SNRPB upregulation functions as an oncogene in hepatocellular carcinoma."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell Biol Int (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/cbin.11307"}], "href": "https://doi.org/10.1002/cbin.11307"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31930637"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31930637"}]}, {"type": "r", "ref": 12, "children": [{"type": "t", "text": "Xiaoyan Wang, Hao Zhang, Zehao Guo, et al. "}, {"type": "b", "children": [{"type": "t", "text": "SNRPB promotes the progression of hepatocellular carcinoma via regulating cell cycle, oxidative stress, and ferroptosis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Aging (Albany NY) (2024)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.18632/aging.205371"}], "href": "https://doi.org/10.18632/aging.205371"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "38189879"}], "href": "https://pubmed.ncbi.nlm.nih.gov/38189879"}]}, {"type": "r", "ref": 13, "children": [{"type": "t", "text": "Y R Li, D Guo, D F Chen, et al. "}, {"type": "b", "children": [{"type": "t", "text": "[Regulatory effect of small nuclear ribonucleoprotein-associated protein B on proliferation and metastasis of liver cancer cells]."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Zhonghua Gan Zang Bing Za Zhi (2022)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.3760/cma.j.cn501113-20200411-00174"}], "href": "https://doi.org/10.3760/cma.j.cn501113-20200411-00174"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "35152671"}], "href": "https://pubmed.ncbi.nlm.nih.gov/35152671"}]}, {"type": "r", "ref": 14, "children": [{"type": "t", "text": "Cory G Toyota, Misty D Davis, Angela M Cosman, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Coilin phosphorylation mediates interaction with SMN and SmB'."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Chromosoma (2010)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1007/s00412-009-0249-x"}], "href": "https://doi.org/10.1007/s00412-009-0249-x"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "19997741"}], "href": "https://pubmed.ncbi.nlm.nih.gov/19997741"}]}, {"type": "r", "ref": 15, "children": [{"type": "t", "text": "Maxim I Maron, Alyssa D Casill, Varun Gupta, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Type I and II PRMTs inversely regulate post-transcriptional intron detention through Sm and CHTOP methylation."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Elife (2022)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.7554/eLife.72867"}], "href": "https://doi.org/10.7554/eLife.72867"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "34984976"}], "href": "https://pubmed.ncbi.nlm.nih.gov/34984976"}]}, {"type": "r", "ref": 16, "children": [{"type": "t", "text": "Alan R Prescott, Alexandra Bales, John James, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Time-resolved quantitative proteomics implicates the core snRNP protein SmB together with SMN in neural trafficking."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Sci (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1242/jcs.137703"}], "href": "https://doi.org/10.1242/jcs.137703"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24357717"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24357717"}]}]}]}