Tudor and KH domain-containing protein (TDRKH)

Tudor and KH domain-containing protein (TDRKH) is a mitochondria-associated RNA-binding protein essential for piRNA (Piwi-interacting RNA) biogenesis and spermatogenesis[1][2][4][7][8]. It functions in the final steps of primary piRNA processing, converting precursor RNAs into mature piRNAs that silence transposable elements in germline cells, thus maintaining genomic integrity during meiosis[1][2][4][7]. TDRKH interacts with PIWI proteins (such as Miwi and Miwi2) and is found in specific germline cytoplasmic granules (pi-bodies and piP-bodies), where it supports the assembly and function of the piRNA pathway[1][7]. Loss of TDRKH results in severe reduction of mature piRNAs, aberrant activation of transposons, and meiotic arrest, leading to male infertility[1][6]. There are no currently known drugs targeting TDRKH, nor is it classified as a traditional druggable therapeutic target such as a receptor or enzyme[2][8].

Other names

TDRKHTDRD2Tudor domain-containing protein 2tudor and KH domain containingputative RNA binding proteinRP11-98D18.8