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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PDLIM3
PDZ and LIM domain 3
Chromosome 4 · 4q35.1
NCBI Gene: 27295Ensembl: ENSG00000154553.17HGNC: HGNC:20767UniProt: A0A087WYF8
37PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cytosolprotein bindingactin bindingstress fiberglomerulonephritisnephrotic syndromeviral pneumoniaresponse to tyrosine kinase inhibitor
✦AI Summary

PDLIM3 encodes a PDZ and LIM domain-containing protein that functions as a cytoskeletal scaffolding protein with diverse roles across multiple cellular contexts. In muscle cells, PDLIM3 localizes to the Z-disc and plays a critical role in muscle structure organization and force transmission between sarcomeres 1. The protein facilitates actin cytoskeleton organization and colocalizes with α-actinin on the Z-line of mature muscle fibers 2. Beyond its structural role, PDLIM3 has emerged as a key regulator of cellular signaling pathways. In sonic hedgehog medulloblastoma, PDLIM3 localizes to primary cilia and supports hedgehog signaling by facilitating ciliogenesis through cholesterol provision, with its PDZ domain mediating cilia localization 3. The protein also regulates YAP-mediated epithelial-mesenchymal transition in head and neck squamous cell carcinoma, where it functions as a tumor suppressor 4. In endometriosis, PDLIM3 promotes cell survival by preventing ferroptosis through stabilization of Gli1 and activation of hedgehog signaling 5. Clinically, PDLIM3 polymorphisms are associated with increased risk of idiopathic dilated cardiomyopathy 1, and elevated PDLIM3 levels serve as biomarkers for poor prognosis in gastric cancer 2 and muscle damage in amyotrophic lateral sclerosis 6.

Sources cited
1
PDLIM3 localizes to Z-disc and polymorphisms increase dilated cardiomyopathy risk
PMID: 31424159
2
PDLIM3 colocalizes with α-actinin and serves as gastric cancer prognostic biomarker
PMID: 35647201
3
PDLIM3 localizes to cilia and supports hedgehog signaling through cholesterol provision
PMID: 36813922
4
PDLIM3 regulates YAP-mediated EMT and functions as tumor suppressor in HNSCC
PMID: 40243891
5
PDLIM3 prevents ferroptosis by stabilizing Gli1 and activating hedgehog signaling
PMID: 38771390
6
PDLIM3 serves as biomarker for muscle damage in ALS
PMID: 41020397
Disease Associationsⓘ20
glomerulonephritisOpen Targets
0.40Weak
nephrotic syndromeOpen Targets
0.33Weak
viral pneumoniaOpen Targets
0.33Weak
response to tyrosine kinase inhibitorOpen Targets
0.28Weak
hypertrophic cardiomyopathyOpen Targets
0.21Weak
dilated cardiomyopathyOpen Targets
0.21Weak
type 2 diabetes mellitusOpen Targets
0.20Weak
Abnormality of the cardiovascular systemOpen Targets
0.16Weak
diabetes mellitusOpen Targets
0.15Weak
bone fractureOpen Targets
0.15Weak
Abruptio PlacentaeOpen Targets
0.14Weak
facial nerve diseaseOpen Targets
0.13Weak
diabetic ketoacidosisOpen Targets
0.12Weak
familial hypertrophic cardiomyopathyOpen Targets
0.11Weak
cardiovascular diseaseOpen Targets
0.11Weak
strokeOpen Targets
0.11Weak
neoplasmOpen Targets
0.10Weak
metabolic syndromeOpen Targets
0.10Suggestive
Alzheimer diseaseOpen Targets
0.10Suggestive
cancerOpen Targets
0.10Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PRICKLE4Shared pathway100%ACTN2Protein interaction97%LDB3Protein interaction82%TTNProtein interaction76%PDLIM2Shared pathway75%PDLIM4Shared pathway75%
Tissue Expression6 tissues
Heart
100%
Lung
25%
Ovary
20%
Brain
5%
Liver
3%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
PDLIM3PRICKLE4ACTN2LDB3TTNPDLIM2PDLIM4
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q53GG5
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.07LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.76 [0.54–1.07]
RankingsWhere PDLIM3 stands among ~20K protein-coding genes
  • #10,667of 20,598
    Most Researched37
  • #10,826of 17,882
    Most Constrained (LOEUF)1.07
Genes detectedPDLIM3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Novel polymorphisms in PDLIM3 and PDLIM5 gene encoding Z-line proteins increase risk of idiopathic dilated cardiomyopathy.
PMID: 31424159
J Cell Mol Med · 2019
1.00
2
PDLIM3 Regulates Migration and Invasion of Head and Neck Squamous Cell Carcinoma via YAP-Mediated Epithelial-Mesenchymal Transition.
PMID: 40243891
Int J Mol Sci · 2025
0.90
3
Ultra-processed foods sourced 7-ketositosterol aggravates colitis through gut dysbiosis induced-PDLIM3 activation.
PMID: 41287530
Gut Microbes · 2025
0.80
4
PDLIM3 supports hedgehog signaling in medulloblastoma by facilitating cilia formation.
PMID: 36813922
Cell Death Differ · 2023
0.70
5
Comprehensive Analysis of PDLIM3 Expression Profile, Prognostic Value, and Correlations with Immune Infiltrates in Gastric Cancer.
PMID: 35647201
J Immunol Res · 2022
0.60