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Target intelligence / Profile preview
Zinc finger MIZ-type containing protein 1 (ZMIZ1)
- Target
- ZMIZ1
- Molecular classification
- Transcription cofactor, Chromatin remodeler, SUMO E3 ligase, Zinc finger protein, PIAS family member
01
Overview
Zinc finger MIZ-type containing protein 1 (ZMIZ1) is a transcriptional coactivator and chromatin remodeler within the PIAS family, possessing a highly conserved MIZ/SUMO-E3-ligase domain and functioning in multi-protein complexes[1][3][6]. It regulates multiple transcription factors—including androgen receptor, p53, Smad3/4, and Notch1—and modulates chromatin accessibility at target gene loci[1][2][3][5][6]. ZMIZ1 plays crucial roles in cell development, proliferation, migration, angiogenesis, and immune regulation, as well as in embryonic, neural, and lymphatic vascular biology[1][2][3][5][6]. Pathogenic variants are linked to neurodevelopmental disorders, cancer, diabetes, autoimmune conditions, and vascular defects[1][2][3]. Its molecular functions make it an attractive candidate for drug development targeting hormone-dependent cancers and autoimmune diseases[3].
- Other names
- Zinc finger MIZ domain-containing protein 1KIAA1224RAI17ZIMP10RP11-519K18.1FLJ13541hZIMP10Zimp10MIZPIAS-like protein Zimp10Retinoic acid-induced protein 17NEDDFSATRAFIP10retinoic acid induced 17Miz1PIAS-like protein on chromosome 10
02
Mechanism of action
Modulation of transcription factors (e.g., androgen receptor, p53, Smad3/4, Notch1); Regulation of chromatin accessibility; SUMOylation activity
03
Biological functions
Transcriptional regulationChromatin remodelingSignal transductionCell proliferationCell migrationEmbryonic developmentAngiogenesisImmune responseNeural and brain developmentLymphatic vascular biology
04
Disease associations
Cancer (including leukemia, hormone-dependent cancers, osteosarcoma)DiabetesMultiple sclerosisNeurodevelopmental disorders (intellectual disability, autism spectrum disorder, ADHD)InflammationCardiovascular disease (via vascular defects and valvulogenesis)Other autoimmune conditions
05
Safety considerations
Genetic perturbation can result in embryonic lethality and vascular defectsDisease-causing mutations are highly intolerant in key functional domains, leading to neurodevelopmental disabilities, abnormal cell proliferation, and tumorigenesis
06
Biomarkers
Mutations/variants in ZMIZ1 (notably in Alanine-rich and TPR domains) may act as biomarkers for neurodevelopmental disorders, leukemia, and other diseases
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