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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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LZTS2
leucine zipper tumor suppressor 2
Chromosome 10 · 10q24.31
NCBI Gene: 84445Ensembl: ENSG00000107816.18HGNC: HGNC:29381UniProt: B4DP66
105PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingvesiclecytosolnegative regulation of canonical Wnt signaling pathwayAbnormality of the skeletal systemneurodegenerative diseasecancerhepatocellular carcinoma
✦AI Summary

LZTS2 (leucine zipper tumor suppressor 2) functions as a multifaceted tumor suppressor through its regulation of the Wnt signaling pathway and centrosomal microtubule dynamics. Primary Function: LZTS2 negatively regulates β-catenin-mediated transcriptional activation by promoting its nuclear exclusion, thereby suppressing the canonical Wnt pathway 123. Additionally, LZTS2 localizes to centrosomes where it negatively regulates CEP135 levels and centrosomal microtubule nucleation 4, and functions as a mechanosensitive effector of α-catenin critical for proper cytokinesis completion 5. Mechanism: LZTS2 stability is controlled by competing ubiquitination and deubiquitination: SPOP and β-TrCP promote LZTS2 degradation via K48-linked polyubiquitination, while HAUSP counteracts this through deubiquitination 26. PLK1-mediated phosphorylation at Ser451 disrupts LZTS2-β-catenin interaction without affecting LZTS2 levels 3. Disease Relevance: LZTS2 downregulation correlates with poor prognosis in hepatocellular carcinoma, colorectal cancer, lung adenocarcinoma, and lung squamous cell carcinoma 12738. Clinical Significance: Peptides disrupting CCDC137-LZTS2 interaction show efficacy in inhibiting HCC progression in organoids and PDX models 1, positioning LZTS2-targeting strategies as promising therapeutic approaches.

Sources cited
1
Primary Function: LZTS2 negatively regulates β-catenin-mediated transcriptional activation by promoting its nuclear exclusion, thereby suppressing the canonical Wnt pathway .
PMID: 38918619
2
Primary Function: LZTS2 negatively regulates β-catenin-mediated transcriptional activation by promoting its nuclear exclusion, thereby suppressing the canonical Wnt pathway .
PMID: 41436424
3
Primary Function: LZTS2 negatively regulates β-catenin-mediated transcriptional activation by promoting its nuclear exclusion, thereby suppressing the canonical Wnt pathway .
PMID: 38740232
4
Additionally, LZTS2 localizes to centrosomes where it negatively regulates CEP135 levels and centrosomal microtubule nucleation , and functions as a mechanosensitive effector of α-catenin critical for proper cytokinesis completion .
PMID: 40521914
5
Additionally, LZTS2 localizes to centrosomes where it negatively regulates CEP135 levels and centrosomal microtubule nucleation , and functions as a mechanosensitive effector of α-catenin critical for proper cytokinesis completion .
PMID: 39786338
6
Mechanism: LZTS2 stability is controlled by competing ubiquitination and deubiquitination: SPOP and β-TrCP promote LZTS2 degradation via K48-linked polyubiquitination, while HAUSP counteracts this through deubiquitination .
PMID: 33420362
7
Disease Relevance: LZTS2 downregulation correlates with poor prognosis in hepatocellular carcinoma, colorectal cancer, lung adenocarcinoma, and lung squamous cell carcinoma .
PMID: 40404727
8
Disease Relevance: LZTS2 downregulation correlates with poor prognosis in hepatocellular carcinoma, colorectal cancer, lung adenocarcinoma, and lung squamous cell carcinoma .
PMID: 40676695
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.46Moderate
neurodegenerative diseaseOpen Targets
0.25Weak
cancerOpen Targets
0.08Suggestive
hepatocellular carcinomaOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.07Suggestive
triple-negative breast cancerOpen Targets
0.07Suggestive
obstructive sleep apneaOpen Targets
0.07Suggestive
laryngeal squamous cell carcinomaOpen Targets
0.06Suggestive
Ochoa syndromeOpen Targets
0.05Suggestive
renal hypodysplasia/aplasia 3Open Targets
0.05Suggestive
caudal duplicationOpen Targets
0.05Suggestive
Mayer-Rokitansky-Kuster-Hauser syndromeOpen Targets
0.05Suggestive
Mayer-Rokitansky-Küster-Hauser syndromeOpen Targets
0.05Suggestive
congenital hydronephrosisOpen Targets
0.05Suggestive
Genetic renal or urinary tract malformationOpen Targets
0.05Suggestive
congenital primary megaureterOpen Targets
0.05Suggestive
Testicular regression syndromeOpen Targets
0.05Suggestive
bladder exstrophyOpen Targets
0.04Suggestive
renal dysplasiaOpen Targets
0.04Suggestive
Hypoparathyroidism - deafness - renal diseaseOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SIPA1L1Protein interaction89%LZTS1Protein interaction84%KATNB1Protein interaction83%KATNA1Protein interaction83%SHANK3Protein interaction81%DYRK1AProtein interaction74%
Tissue Expression6 tissues
Ovary
100%
Lung
62%
Heart
44%
Bone Marrow
35%
Brain
27%
Liver
24%
Gene Interaction Network
Click a node to explore
LZTS2SIPA1L1LZTS1KATNB1KATNA1SHANK3DYRK1A
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9BRK4
View on AlphaFold ↗
RankingsWhere LZTS2 stands among ~20K protein-coding genes
  • #4,540of 20,598
    Most Researched105 · top quartile
Genes detectedLZTS2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Disrupting CCDC137-mediated LZTS2 and β-TrCP interaction in the nucleus inhibits hepatocellular carcinoma development via β-catenin and AKT.
PMID: 38918619
Cell Death Differ · 2025
1.00
2
SPOP and HAUSP bidirectionally regulate LZTS2 ubiquitination to modulate the Wnt pathway.
PMID: 41436424
Cell Death Dis · 2025
0.90
3
LZTS2 methylation as a potential diagnostic and prognostic marker in LIHC and STAD: Evidence from bioinformatics and in vitro analyses.
PMID: 40404727
Sci Rep · 2025
0.80
4
α-Catenin force-sensitive binding and sequestration of LZTS2 leads to cytokinesis failure.
PMID: 39786338
J Cell Biol · 2025
0.70
5
LZTS2 Negatively Regulates Centrosomal CEP135 Levels and Microtubule Nucleation.
PMID: 40521914
Cytoskeleton (Hoboken) · 2026
0.60