KLHDC3
Description
The KLHDC3 (kelch domain containing 3) is a protein-coding gene located on chromosome 6.
KLHDC3, or Kelch domain-containing protein 3, is a protein encoded by the KLHDC3 gene in humans. The protein comprises six repeated kelch motifs structurally similar to RAG2, a protein involved in V(D)J recombination. KLHDC3 is specifically expressed in the testis, with its localization in pachytene spermatocytes found in both the cytoplasm and meiotic chromatin. This suggests a potential role for KLHDC3 in meiotic recombination.
KLHDC3 is a substrate-recognition component of a Cul2-RING (CRL2) E3 ubiquitin-protein ligase complex, specifically within the DesCEND (destruction via C-end degrons) pathway. This complex recognizes a C-degron, a motif of less than ten residues located at the extreme C-terminus of target proteins. This recognition leads to the ubiquitination and degradation of the target proteins. The DesCEND pathway preferentially targets proteins with a glycine (Gly) at their C-terminus, specifically recognizing the C-terminal -Arg-(Xaa)n-Arg-Gly, -Arg-(Xaa)n-Lys-Gly, and -Arg-(Xaa)n-Gln-Gly degrons. KLHDC3 plays a role in the degradation of truncated SELENOV and SEPHS2 selenoproteins that result from failed UGA/Sec decoding. These truncated selenoproteins possess a glycine at their C-terminus. KLHDC3 may also be involved in meiotic recombination.
KLHDC3 is also known as PEAS, dJ20C7.3.
