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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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KLHL21
kelch like family member 21
Chromosome 1 · 1p36.31
NCBI Gene: 9903Ensembl: ENSG00000162413.17HGNC: HGNC:29041UniProt: Q9UJP4
37PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
centrosomeCul3-RING ubiquitin ligase complexpolar microtubuleprotein bindinghypertensiontype 2 diabetes mellitusdiabetes mellitusresponse to xenobiotic stimulus
✦AI Summary

KLHL21 (kelch-like family member 21) is a substrate-specific adapter of the Cul3-based E3 ubiquitin ligase complex (BCR(KLHL21)) that regulates multiple cellular processes. Functionally, KLHL21 is required for efficient chromosome 1 and cytokinesis by controlling translocation of the chr1 passenger complex (CPC) from chr1 to the spindle midzone during anaphase 1. The complex mediates ubiquitination of aurora B kinase (AURKB), though this modification may not lead to proteasomal degradation 1. Clinically, KLHL21 exhibits tumor-suppressive functions across multiple cancer types. In gastric cancer, KLHL21 loss promotes STAT3 activation through PABPC1-mediated PIK3CB translational enhancement, driving metaplasia-to-dysplasia progression and poor survival outcomes 2. In hepatocellular carcinoma, KLHL21 upregulation correlates with cancer progression and serves as a potential therapeutic target 3. Conversely, in breast cancer, KLHL21 downregulation reduces KLHL21-mediated IKKβ binding, allowing NF-κB p65 pathway activation and promoting metastasis 4. Similarly, in cholangiocarcinoma and bladder cancer, KLHL21 inhibition accelerates proliferation via ERK pathway activation and NF-κB signaling respectively 5, 6. Recently, KLHL21 was identified among prognostic gene signatures for hepatocellular carcinoma 7 and in ubiquitination-related diagnostic signatures for Alzheimer's disease 8, suggesting broader pathological relevance beyond cancer.

Sources cited
1
KLHL21 is a Cul3 adapter required for chromosome alignment, cytokinesis, and CPC translocation to spindle midzone; directly binds and ubiquitinates aurora B
PMID: 19995937
2
KLHL21 loss in gastric cancer promotes STAT3 activation through PABPC1-mediated PIK3CB translation, driving metaplasia progression and poor survival
PMID: 38969490
3
KLHL21 upregulation in hepatocellular carcinoma; KLHL21 knockdown inhibits HCC cell proliferation, migration and invasion
PMID: 27769251
4
Macrophage-derived extracellular vesicle miR-660 targets KLHL21 in breast cancer, reducing KLHL21-IKKβ binding and activating NF-κB p65 pathway
PMID: 35084622
5
KLHL21 inhibition in cholangiocarcinoma reduces proliferation, triggers G0/G1 arrest, and decreases ERK1/2 phosphorylation
PMID: 29574153
6
KLHL21 suppresses bladder cancer progression by stabilizing CYLD, preventing its ubiquitination and degradation, thereby inhibiting NF-κB signaling
PMID: 36538852
7
KLHL21 identified as prognostic feature in hepatocellular carcinoma crotonylation gene signature
PMID: 39428564
8
KLHL21 identified among ubiquitination-related genes with diagnostic implications for Alzheimer's disease
PMID: 38730027
Disease Associationsⓘ20
hypertensionOpen Targets
0.43Moderate
type 2 diabetes mellitusOpen Targets
0.36Weak
diabetes mellitusOpen Targets
0.34Weak
response to xenobiotic stimulusOpen Targets
0.31Weak
obesityOpen Targets
0.27Weak
overnutritionOpen Targets
0.22Weak
essential hypertensionOpen Targets
0.12Weak
ependymomaOpen Targets
0.11Weak
metabolic syndromeOpen Targets
0.09Suggestive
Increased blood pressureOpen Targets
0.07Suggestive
hepatocellular carcinomaOpen Targets
0.05Suggestive
sleep apneaOpen Targets
0.04Suggestive
cardiovascular diseaseOpen Targets
0.03Suggestive
obstructive sleep apneaOpen Targets
0.03Suggestive
breast cancerOpen Targets
0.03Suggestive
cholangiocarcinomaOpen Targets
0.02Suggestive
uveitisOpen Targets
0.02Suggestive
neoplasmOpen Targets
0.02Suggestive
urinary bladder carcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RBX1Protein interaction99%KLHL9Protein interaction95%KLHL13Protein interaction94%KLHL22Protein interaction93%KLHL42Protein interaction92%KBTBD8Protein interaction92%
Tissue Expression6 tissues
Heart
100%
Ovary
72%
Lung
53%
Liver
46%
Bone Marrow
38%
Brain
32%
Gene Interaction Network
Click a node to explore
KLHL21RBX1KLHL9KLHL13KLHL22KLHL42KBTBD8
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9UJP4
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.65LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.29 [1.01–1.65]
RankingsWhere KLHL21 stands among ~20K protein-coding genes
  • #10,641of 20,598
    Most Researched37
  • #15,887of 17,882
    Most Constrained (LOEUF)1.65
Genes detectedKLHL21
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
KLHL21 suppresses gastric tumourigenesis via maintaining STAT3 signalling equilibrium in stomach homoeostasis.
PMID: 38969490
Gut · 2024
1.00
2
KLHL21, a novel gene that contributes to the progression of hepatocellular carcinoma.
PMID: 27769251
BMC Cancer · 2016
0.90
3
Tumor-promoting mechanisms of macrophage-derived extracellular vesicles-enclosed microRNA-660 in breast cancer progression.
PMID: 35084622
Breast Cancer Res Treat · 2022
0.80
4
Inhibition of KLHL21 prevents cholangiocarcinoma progression through regulating cell proliferation and motility, arresting cell cycle and reducing Erk activation.
PMID: 29574153
Biochem Biophys Res Commun · 2018
0.70
5
Integrative transcriptome analysis identifies a crotonylation gene signature for predicting prognosis and drug sensitivity in hepatocellular carcinoma.
PMID: 39428564
J Cell Mol Med · 2024
0.60