ZNF142 encodes a zinc finger transcription factor containing 31 C2H2-type zinc finger domains that functions as a DNA-binding transcription factor regulating RNA polymerase II-mediated gene expression 1. The protein localizes to the nucleus where it participates in transcriptional regulation and potentially chr2 organization 2. Biallelic loss-of-function variants in ZNF142 cause neurodevelopmental disorder with impaired speech and hyperkinetic movements (NEDISHM), an autosomal recessive condition 1. ZNF142 deficiency produces a distinctive DNA methylation signature affecting a limited number of genomic loci, with 88 differentially methylated probes identified in affected individuals, suggesting ZNF142 regulates methylation at specific regulatory regions 2. Clinically, ZNF142 mutations lead to syndromic neurodevelopmental disorder characterized by mild-to-moderate intellectual disability, speech impairment, seizures (often early-onset), hypotonia, movement disorders including dystonia and ataxia, developmental delay, behavioral features, and facial dysmorphism 34. Frameshift and nonsense variants appear more common than missense mutations among identified pathogenic variants 3. Expression studies demonstrate that pathogenic variants reduce ZNF142 mRNA levels substantially 5. The phenotypic variability observed among affected individuals, including identical twins carrying identical mutations, indicates that ZNF142 pathogenic variants cause complex neurodevelopmental dysfunction through transcriptional dysregulation 5.