ZNF100 is a zinc finger protein that functions as a DNA-binding transcription factor with RNA polymerase II specificity 1. The protein acts as a sequence-specific repressor of THE1A retrotransposons, having evolved during the simian lineage to specifically silence this retrovirus family through contact residue changes in its zinc finger domain that confer preferential binding to THE1A elements 1. ZNF100 demonstrates involvement in disease pathogenesis across multiple contexts. It was identified as a potential drug target in primary aldosteronism through integrated bioinformatics analysis of transcriptome-wide association studies and Mendelian randomization 2. Expression variation of ZNF100 has been associated with ovarian cancer patient outcomes, with regulatory elements interacting with its promoter region showing correlation with candidate outcome variants at the 19p12 locus 3. Additionally, genetic variants within ZNF100-like regions on chromosome 19 were identified as significantly associated with pancreatic cancer susceptibility in Jewish populations 4. A long non-coding RNA transcript designated ZNF100-6:2 shows promise as a diagnostic biomarker for active pulmonary tuberculosis, with high diagnostic accuracy (ROC AUC = 0.9796) and downregulation in affected patients 5. ZNF100 represents a primate-specific zinc finger gene with both regulatory and disease-relevant functions.