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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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KLHDC10
kelch domain containing 10
Chromosome 7 · 7q32.2
NCBI Gene: 23008Ensembl: ENSG00000128607.15HGNC: HGNC:22194UniProt: Q6PID8
34PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingubiquitin-like ligase-substrate adaptor activityCul2-RING ubiquitin ligase complexcytoplasmHypercholesterolemiapsoriasisAlzheimer diseaseosteoarthritis, hip
✦AI Summary

KLHDC10 (kelch domain containing 10) functions as a substrate-recognition component of the CRL2 (Cul2-RING) E3 ubiquitin ligase complex, which targets proteins for ubiquitin-dependent degradation via the DesCEND pathway 12. The complex specifically recognizes C-degrons—short peptide motifs at protein C-termini, particularly proline-glycine or alanine tails (CAT tails)—leading to substrate ubiquitination 34. KLHDC10 possesses a U-shaped degron-binding pocket with conformational malleability that enables recognition of varying C-terminal sequences, distinguishing it from related KLHDC family members 4. A critical function involves ribosome-associated quality control (RQC), where CRL2(KLHDC10) acts downstream of NEMF to recognize and degrade incompletely synthesized nascent chains bearing alanine tails from stalled ribosomes 25. Beyond RQC, KLHDC10 participates in oxidative stress responses by inhibiting protein phosphatase 5 (PP5) activity on MAP3K5, thereby promoting sustained ASK1 activation and stress-induced cell death through MAPK pathways 6. Disease relevance is indicated by association with colorectal cancer risk through DNA methylation regulation 7. These functions position KLHDC10 as a key regulator of protein quality control and cellular stress responses.

Sources cited
1
KLHDC10 recognizes alanine-tailed ribosome stalling products in RQC pathway
PMID: 33909987
2
KLHDC10 directly binds alanine-tail C-degrons with specific binding pocket
PMID: 37676773
3
KLHDC10 has conformational malleability for C-degron recognition compared to other KLHDC family members
PMID: 39548056
4
RQC mechanisms and NEMF function in sensing stalled ribosomes
PMID: 35452614
5
KLHDC10 suppresses PP5 phosphatase activity on ASK1 in oxidative stress response
PMID: 23102700
6
KLHDC10 associated with colorectal cancer risk through DNA methylation regulation
PMID: 35247911
Disease Associationsⓘ20
HypercholesterolemiaOpen Targets
0.32Weak
psoriasisOpen Targets
0.28Weak
Alzheimer diseaseOpen Targets
0.28Weak
osteoarthritis, hipOpen Targets
0.27Weak
MODYOpen Targets
0.05Suggestive
systemic inflammatory response syndromeOpen Targets
0.04Suggestive
diabetes mellitus, permanent neonatal 4Open Targets
0.04Suggestive
attention deficit hyperactivity disorderOpen Targets
0.04Suggestive
hereditary attention deficit-hyperactivity disorderOpen Targets
0.04Suggestive
maturity-onset diabetes of the young type 13Open Targets
0.04Suggestive
maturity-onset diabetes of the young type 3Open Targets
0.04Suggestive
permanent neonatal diabetes mellitus 1Open Targets
0.04Suggestive
diabetes mellitus, transient neonatal, 3Open Targets
0.04Suggestive
maturity-onset diabetes of the young type 10Open Targets
0.03Suggestive
hyper-IgE recurrent infection syndrome 5, autosomal recessiveOpen Targets
0.03Suggestive
coronary artery diseaseOpen Targets
0.02Suggestive
neuroendocrine neoplasmOpen Targets
0.02Suggestive
Increased total eosinophil countOpen Targets
0.02Suggestive
Abnormal sperm morphologyOpen Targets
0.02Suggestive
infectionOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
FEM1CProtein interaction94%PRAMEF6Protein interaction94%ELOBProtein interaction93%FEM1BProtein interaction93%ZER1Protein interaction93%APPBP2Protein interaction93%
Tissue Expression6 tissues
Brain
100%
Liver
69%
Heart
61%
Lung
40%
Ovary
38%
Bone Marrow
28%
Gene Interaction Network
Click a node to explore
KLHDC10FEM1CPRAMEF6ELOBFEM1BZER1APPBP2
PROTEIN STRUCTURE
Preparing viewer…
PDB9D8P · 3.20 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.53Moderately Constrained
pLIⓘ
0.95Intolerant
Observed/Expected LoF0.33 [0.21–0.53]
RankingsWhere KLHDC10 stands among ~20K protein-coding genes
  • #11,176of 20,598
    Most Researched34
  • #3,310of 17,882
    Most Constrained (LOEUF)0.53 · top quartile
Genes detectedKLHDC10
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Ribosome-associated quality-control mechanisms from bacteria to humans.
PMID: 35452614
Mol Cell · 2022
1.00
2
Mechanism and evolutionary origins of alanine-tail C-degron recognition by E3 ligases Pirh2 and CRL2-KLHDC10.
PMID: 37676773
Cell Rep · 2023
0.90
3
Structural basis for C-degron selectivity across KLHDCX family E3 ubiquitin ligases.
PMID: 39548056
Nat Commun · 2024
0.80
4
Principles of paralog-specific targeted protein degradation engaging the C-degron E3 KLHDC2.
PMID: 39396041
Nat Commun · 2024
0.70
5
Genetic Regulation of DNA Methylation Yields Novel Discoveries in GWAS of Colorectal Cancer.
PMID: 35247911
Cancer Epidemiol Biomarkers Prev · 2022
0.60