TEX13B is a testis-expressed gene located on the X chromosome X functions as a transcriptional repressor involved in male germ cell development. The protein exhibits transcriptional repressor activity 1, with this repressive function residing in a conserved ~145 amino acid domain shared across the TEX13 protein family 1. TEX13B plays a critical role in metabolic reprogramming during spermatogenesis by regulating oxidative phosphorylation (OXPHOS) complex expression; TEX13B knockout leads to downregulation of OXPHOS complexes and compensatory upregulation of glycolytic genes 2. Regarding disease relevance, TEX13B variants have been identified in infertile men with non-obstructive azoospermia, including a rare variant (p.Gly237Glu) found exclusively in azoospermic patients 2. However, recent evidence suggests TEX13B loss-of-function variants may not be monogenic culprits for male infertility, as hemizygous loss-of-function variants were also detected in fertile control men, contradicting earlier disease associations 3. X-autosomal translocations disrupting TEX13B have been clinically associated with azoospermia 4. Despite these associations, TEX13B's role in infertility genetics requires further investigation, and screening for TEX13B variants should be interpreted cautiously in genetic diagnostics of azoospermic men.