KLHDC10
Description
The KLHDC10 (kelch domain containing 10) is a protein-coding gene located on chromosome 7.
KLHDC10 is a substrate-recognition component of a Cul2-RING (CRL2) E3 ubiquitin-protein ligase complex within the DesCEND (destruction via C-end degrons) pathway. It identifies a C-degron, a motif of less than ten residues, at the extreme C-terminus of target proteins, leading to their ubiquitination and degradation. This C-degron can be present in full-length proteins, truncated proteins, or proteolytically cleaved forms. The CRL2(KLHDC10) complex specifically targets proteins with a proline-glycine (Pro-Gly) or an alanine tail (CAT tail) at the C-terminus, resulting in their ubiquitination and subsequent degradation. Additionally, this complex participates in the ribosome-associated quality control (RQC) pathway, recognizing CAT tails associated with stalled nascent chains, leading to their ubiquitination and degradation. Furthermore, KLHDC10 contributes to oxidative stress-induced cell death by activating MAP3K5, and inhibits PPP5C phosphatase activity on MAP3K5. It also acts as a regulator of necroptosis. KLHDC10 is a component of a CRL2 E3 ubiquitin-protein ligase complex, also known as the ECS (Elongin BC-CUL2/5-SOCS-box protein) complex, which is composed of CUL2, Elongin BC (ELOB and ELOC), RBX1, and the substrate-specific adapter KLHDC10.
KLHDC10 is also known as PNAS-138, slim.
Associated Diseases
- diabetes mellitus, permanent neonatal 4
- diabetes mellitus, transient neonatal, 3
- permanent neonatal diabetes mellitus 1
