ArticleBMC genomics2013
Loss of WSTF results in spontaneous fluctuations of heterochromatin formation and resolution, combined with substantial changes to gene expression.
Article in BMC genomics, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 25 citations in OpenAlex.
- Epigenetic regulation of craniofacial development and disease.Birth defects research · 2024Review
- Loss of Baz1b in mice causes perinatal lethality, growth failure, and variable multi-system outcomes.Developmental biology · 2024Article
- DNA methylation profiles in individuals with rare, atypical 7q11.23 CNVs correlate with GTF2I and GTF2IRD1 copy number.NPJ genomic medicine · 2023Article
- In individuals with Williams syndrome, dysregulation of methylation in non-coding regions of neuronal and oligodendrocyte DNA is associated with pathology and cortical development.Molecular psychiatry · 2023Article
- Interplay between chromatin marks in development and disease.Nature reviews. Genetics · 2022Review
- Downregulation of Williams syndrome transcription factor (WSTF) suppresses glioblastoma cell growth and invasion by inhibiting PI3K/AKT signal pathway.European journal of histochemistry : EJH · 2021Article
- BAZ1B the Protean Protein.Genes · 2021Review
- [Research Progress of Epigenetics in Pathogenesis and Treatment of Malignant Tumors].Zhongguo fei ai za zhi = Chinese journal of lung cancer · 2020Review
- Dosage analysis of the 7q11.23 Williams region identifiesScience advances · 2019Article
- Quantitative Proteomics of the Mitotic Chromosome Scaffold Reveals the Association of BAZ1B with Chromosomal Axes.Molecular & cellular proteomics : MCP · 2019Article
- Loss of SETDB1 decompacts the inactive X chromosome in part through reactivation of an enhancer in the IL1RAPL1 gene.Epigenetics & chromatin · 2018Article
- Chromatin remodelling and epigenetic state regulation by non-coding RNAs in the diseased heart.Non-coding RNA research · 2018Review
- Linking replication stress with heterochromatin formation.Chromosoma · 2016Review
- BAZ1B in Nucleus Accumbens Regulates Reward-Related Behaviors in Response to Distinct Emotional Stimuli.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Article
- Symmetrical Dose-Dependent DNA-Methylation Profiles in Children with Deletion or Duplication of 7q11.23.American journal of human genetics · 2015Article
- The B-WICH chromatin-remodelling complex regulates RNA polymerase III transcription by promoting Max-dependent c-Myc binding.Nucleic acids research · 2015Article
- BAZ1B is dispensable for H2AX phosphorylation on Tyrosine 142 during spermatogenesis.Biology open · 2015Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
backgroundWilliams syndrome transcription factor (WSTF) is a multifaceted protein that is involved in several nuclear processes, including replication, transcription, and the DNA damage response. WSTF participates in a chromatin-remodeling complex with the ISWI ATPase, SNF2H, and is thought to contribute to the maintenance of heterochromatin, including at the human inactive X chromosome (Xi). WSTF is encoded by BAZ1B, and is one of twenty-eight genes that are hemizygously deleted in the genetic disorder Williams-Beuren syndrome (WBS).
resultsTo explore the function of WSTF, we performed zinc finger nuclease-assisted targeting of the BAZ1B gene and isolated several independent knockout clones in human cells. Our results show that, while heterochromatin at the Xi is unaltered, new inappropriate areas of heterochromatin spontaneously form and resolve throughout the nucleus, appearing as large DAPI-dense staining blocks, defined by histone H3 lysine-9 trimethylation and association of the proteins heterochromatin protein 1 and structural maintenance of chromosomes flexible hinge domain containing 1. In three independent mutants, the expression of a large number of genes were impacted, both up and down, by WSTF loss.
conclusionsGiven the inappropriate appearance of regions of heterochromatin in BAZ1B knockout cells, it is evident that WSTF performs a critical role in maintaining chromatin and transcriptional states, a property that is likely compromised by WSTF haploinsufficiency in WBS patients.
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