4.7 Review

ZMIZ proteins: partners in transcriptional regulation and risk factors for human disease

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Summary: ZMIZ1, a transcriptional coactivator related to the PIAS family, regulates the activity of various transcription factors and selectively interacts with Notch1 to influence T cell development and leukemogenesis in mammals. In zebrafish, CRISPR/Cas9 mutations in the zmiz1a gene led to lethality and delayed erythroid maturation, with dysregulation of autophagy gene expression and impaired response to vitamin D. These findings suggest a role of Zmiz1a beyond red blood cell differentiation in the regulation of autophagy genes and mediation of vitamin D activity.

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