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Article

HELLS and CDCA7 comprise a bipartite nucleosome remodeling complex defective in ICF syndrome.

Proceedings of the National Academy of Sciences of the United States of America - 30 Jan 2018

Jenness Christopher, Giunta Simona, Müller Manuel M, Kimura Hiroshi, Muir Tom W, Funabiki Hironori

Abstract excerpt

Mutations in CDCA7, the SNF2 family protein HELLS (LSH), or the DNA methyltransferase DNMT3b cause immunodeficiency-centromeric instability-facial anomalies (ICF) syndrome. While it has been speculated that DNA methylation defects cause this disease, little is known about the molecular function of CDCA7 and its functional relationship to HELLS and DNMT3b. Systematic analysis of how the cell cycle, H3K9...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Open in PubMed Open full text

Identifiers

PMID: 29339483
DOI: 10.1073/pnas.1717509115
PMCID: PMC5798369

Genes

CDCA7
HELLS

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

HELLS and CDCA7 comprise a bipartite nucleosome remodeling complex defective in ICF syndrome.PMID 29339483Read ↗

CDCA7 is a hemimethylated DNA adaptor for the nucleosome remodeler HELLSSemantic proximity Read ↗

Coevolution of the CDCA7-HELLS ICF-related nucleosome remodeling complex and DNA methyltransferasesSemantic proximity Read ↗

CDCA7 and HELLS suppress DNA:RNA hybrid-associated DNA damage at pericentromeric repeatsSemantic proximity Read ↗

The C-terminal 4CXXC-type zinc finger domain of CDCA7 recognizes hemimethylated DNA and modulates activities of chromatin remodeling enzyme HELLS.Semantic proximity Read ↗

Comparative methylome analysis of ICF patients identifies heterochromatin loci that require ZBTB24, CDCA7 and HELLS for their methylated state.Semantic proximity Read ↗

DNA methylation in disease: Immunodeficiency, Centromeric instability, Facial anomalies syndrome.Semantic proximity Read ↗

Chromatin remodeling in replication‐uncoupled maintenance DNA methylation and chromosome stability: Insights from ICF syndrome studiesSemantic proximity Read ↗

The ICF syndrome protein CDCA7 harbors a unique DNA binding domain that recognizes a CpG dyad in the context of a non-B DNA.Semantic proximity Read ↗

Investigation into the role of LSH ATPase in chromatin remodellingSemantic proximity Read ↗

Genome-wide DNA methylation patterns in LSH mutant reveals de-repression of repeat elements and redundant epigenetic silencing pathways.Semantic proximity Read ↗

Serum-dependent recruitment of the chromatin remodeler CHD8 to promoters is mediated by the ERK-ELK pathwaySemantic proximity Read ↗

Mutations in CDCA7 and HELLS cause immunodeficiency-centromeric instability-facial anomalies syndrome.Semantic proximity Read ↗

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