Target intelligence / Profile preview

Zinc finger protein 57 homolog (ZFP57)

Target
ZFP57
Molecular classification
Transcription factor, KRAB zinc finger protein, C2H2-type zinc-finger protein family
01

Overview

Zinc finger protein 57 homolog (ZFP57) is a member of the KRAB-containing C2H2-type zinc finger protein family that acts as a sequence-specific DNA-binding transcriptional repressor. It plays a crucial role in the establishment and maintenance of genomic imprinting, primarily by recognizing methylated CpG dinucleotides at specific hexamer motifs, notably within imprinting control regions. ZFP57 recruits the KAP1 co-repressor complex to these sites, which leads to the establishment and maintenance of repressive chromatin marks—specifically H3K9me3—thus maintaining parental-origin-specific DNA methylation patterns during early development. Mutations in ZFP57 disrupt these processes and are associated with transient neonatal diabetes mellitus and multi-locus imprinting disturbances. While ZFP57 is essential for imprint maintenance in mice, in humans, it works in coordination with other KRAB zinc finger proteins, such as ZNF445.

Other names
Zinc finger protein 57ZFP57C6orf40ZNF698zinc finger protein 698TNDM1bA145L22bA145L22.2Zfp-57
02

Biological functions

Maintenance of genomic imprintingTranscriptional repressionDNA methylation regulationChromatin modification
03

Disease associations

Transient neonatal diabetes mellitus (TNDM1)Multi-locus imprinting disturbance (MLID)Other imprinting disorders
04

Safety considerations

Loss of function can lead to hypomethylation and imprinting disorders such as TNDM1 and MLID
05

Biomarkers

Methylation status at imprinted loci (especially PLAGL1 DMR)

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