TDRKH

TDRKH (Tudor And KH Domain Containing) is a Protein Coding gene. Diseases related to TDRKH include Ewing Sarcoma. Among its related pathways are Mitotic Prophase and Gene Expression. Gene Ontology (GO) annotations related to TDRKH include nucleic acid binding and RNA binding. An important paralog of TDRKH is TDRD5.

Full Name

TDRKH

Function

Participates in the primary piRNA biogenesis pathway and is required during spermatogenesis to repress transposable elements and prevent their mobilization, which is essential for the germline integrity. The piRNA metabolic process mediates the repression of transposable elements during meiosis by forming complexes composed of piRNAs and Piwi proteins and govern the methylation and subsequent repression of transposons. Required for the final steps of primary piRNA biogenesis by participating in the processing of 31-37 nt intermediates into mature piRNAs. May act in pi-bodies and piP-bodies by transferring piRNA precursors or intermediates to or between these granules.

Biological Process

Biological Process DNA methylation involved in gamete generationISS:UniProtKB
Biological Process fertilizationISS:UniProtKB
Biological Process gene silencing by RNAIEA:UniProtKB-KW
Biological Process male meiotic nuclear divisionISS:UniProtKB
Biological Process P granule organizationIBA:GO_Central1 Publication
Biological Process piRNA metabolic processISS:UniProtKB
Biological Process spermatogenesisISS:UniProtKB

Cellular Location

Cytoplasm
Mitochondrion
Probable component of the meiotic nuage, also named P granule, a germ-cell-specific organelle required to repress transposon activity during meiosis. Colocalizes with pi- and piP-bodies, a subset of the nuage which contains secondary piRNAs. Associated with mitochondria in the germline.

PTM

Ubiquitinated by PRKN during mitophagy, leading to its degradation and enhancement of mitophagy. Deubiquitinated by USP30.