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Article

NIPBL Controls RNA Biogenesis to Prevent Activation of the Stress Kinase PKR.

Cell reports - 5 Jan 2016

Yuen Kobe C, Xu Baoshan, Krantz Ian D, Gerton Jennifer L

Abstract excerpt

NIPBL, a cohesin loader, has been implicated in transcriptional control and genome organization. Mutations in NIPBL, cohesin, and its deacetylase HDAC8 result in Cornelia de Lange syndrome. We report activation of the RNA-sensing kinase PKR in human lymphoblastoid cell lines carrying NIPBL or HDAC8 mutations, but not SMC1A or SMC3 mutations. PKR activation can be triggered by unmodified RNAs. Gene expression...

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Source

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Identifiers

PMID: 26725122
DOI: 10.1016/j.celrep.2015.12.012
PMCID: PMC4904785

Genes

NIPBL
HDAC8
SMC3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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NIPBL Controls RNA Biogenesis to Prevent Activation of the Stress Kinase PKR.PMID 26725122Read ↗

Regulation of the cohesin-loading factor NIPBL: Role of the lncRNA NIPBL-AS1 and identification of a distal enhancer element.Semantic proximity Read ↗

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The cohesin loader NIPBL interacts with pre-ribosomal RNA and treacle to regulate ribosomal RNA synthesisSemantic proximity Read ↗

Mutant cohesin affects RNA polymerase II regulation in Cornelia de Lange syndrome.Semantic proximity Read ↗

Decreasing Wapl dosage partially corrects embryonic growth and brain transcriptome phenotypes in Nipbl+/- embryos.Semantic proximity Read ↗

<i>MAU2</i> and <i>NIPBL</i> variants in Cornelia de Lange syndrome reveal MAU2-independent loading of cohesin and uncover a protective mechanism against early truncating mutations in NIPBLSemantic proximity Read ↗

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