Zinc Finger and BTB Domain Containing 24 Proteins (ZBTB24)

Human Zinc Finger and BTB Domain Containing 24 (ZBTB24) interaction partners

1. Here, we performed a comparative analysis of perturbed DNA methylation landscapes in a cohort of ICF patients using an array-based assay to (i) evaluate the similarities and differences in methylation landscapes depending on patient genotypes as an index of a functional link between the four ICF factors, (ii) identify genomic regions that rely on DNMT3B, ZBTB24, CDCA7 or HELLS for their methylation status

2. data indicate that SRRM4 regulates alternative RNA splicing of the Bif-1 gene that enables PCa cells resistant to apoptotic stimuli under anti-cancer therapies, and may contribute to AdPC progression into t-NEPC.

3. The biallelic deletion of ZBTB24 provides strong support for the hypothesis that most ICF2 patients suffer from a ZBTB24 loss of function mechanism and confirms that complete absence of ZBTB24 is compatible with human life. This is in contrast to the observed early embryonic lethality in mice lacking functional Zbtb24

4. Transcriptome analysis identified Cdca7 as the top down-regulated gene in Zbtb24 homozygous mutant mESCs, which can be restored by ectopic ZBTB24 expression. Finally, we show that this regulation is conserved between species and that CDCA7 levels are reduced in patients carrying ZBTB24 nonsense mutations

5. Downregulation of ZBTB24 increases the expression of IRF-4 and reduces b-cell proliferation.

6. Our results demonstrate a novel role of Bif-1 in hepatocellular carcinoma (HCC), in which Bif-1 promotes cell metastasis by regulating Cdc42 expression and activity.

7. ICF2, caused by biallelic ZBTB24 gene mutations, is acknowledged primarily as an isolated B-cell defect.

8. This suggests that Bif-1 protein expression may be a useful prognostic marker in early-stage CRC

9. Clinical and genetic data of mutations in DNMT3B and ZBTB24 in patients with ICF syndrome, were compared.

10. We report three novel ZBTB24 mutations in Japanese and Cape Verdean type 2 ICF syndrome patients.

11. A novel deletion in the ZBTB24 gene was responsible for causing immunodeficiency, centromeric instability and facial anomalies syndrome type 2 in this family.

12. These data indicate that ZBTB24 is involved in DNA methylation of juxtacentromeric DNA and in B cell development and/or B and T cell interactions.

Mouse (Murine) Zinc Finger and BTB Domain Containing 24 (ZBTB24) interaction partners

1. The biallelic deletion of ZBTB24 provides strong support for the hypothesis that most ICF2 patients suffer from a ZBTB24 loss of function mechanism and confirms that complete absence of ZBTB24 is compatible with human life. This is in contrast to the observed early embryonic lethality in mice lacking functional Zbtb24

2. Transcriptome analysis identified Cdca7 as the top down-regulated gene in Zbtb24 homozygous mutant mESCs, which can be restored by ectopic ZBTB24 expression. Finally, we show that this regulation is conserved between species and that CDCA7 levels are reduced in patients carrying ZBTB24 nonsense mutations

3. mutation of human Zbtb24 causes ICF syndrome

4. consistent induction of ZNF450 by BMP-2 after 4 h in three murine pluripotent cell lines suggests that ZNF450 may play a role in the BMP-2 signalling pathway