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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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FHL3
four and a half LIM domains 3
Chromosome 1 · 1p34.3
NCBI Gene: 2275Ensembl: ENSG00000183386.12HGNC: HGNC:3704UniProt: Q13643
91PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingfocal adhesionactin cytoskeleton organizationstress fiberneurodegenerative diseasecoronary artery diseaseandrogenetic alopeciaimmune system disease
✦AI Summary

FHL3 (four and a half LIM domains 3) is a multifunctional LIM domain protein involved in transcriptional regulation, muscle development, and cellular signaling. As a transcriptional coactivator, FHL3 is recruited by SOX15 to FOXK1 promoters and can also function as a co-repressor, exemplified by its interaction with MZF-1 to suppress human high-affinity IgE receptor beta-chain gene expression 1. In muscle biology, FHL3 plays crucial roles in myogenesis and muscle organ development, with studies identifying it as a biomarker for muscle function improvement in myotonic dystrophy type 1 patients 2. The protein demonstrates diverse cellular effects, including inhibition of HepG2 cell growth while promoting ECV304 cell proliferation and increasing angiogenin gene expression 3. FHL3 participates in oncogenic pathways, particularly in hepatocellular carcinoma where it interacts with MAZ to promote KRAS transcription downstream of Hippo-YAP signaling 4. Additionally, FHL3 mediates cardiovascular protection by competitively binding to prevent ubiquitin-proteasome degradation of phosphorylated ERK1/2 and JNK, thereby inhibiting aortic valve calcification when disrupted by AMBP 5. The protein has also been identified as part of prognostic models in cancer and immune-related disorders 67.

Sources cited
1
FHL3 acts as transcriptional co-repressor by binding MZF-1 to suppress IgE receptor beta-chain gene expression
PMID: 15453830
2
FHL3 involvement in muscle organ development and as biomarker for muscle function improvement in myotonic dystrophy
PMID: 37694373
3
FHL3 inhibits HepG2 cell growth, promotes ECV304 cell proliferation, and increases angiogenin expression
PMID: 19547926
4
FHL3 interacts with MAZ to promote KRAS transcription downstream of Hippo-YAP signaling in hepatocellular carcinoma
PMID: 41184244
5
FHL3 binding prevents ubiquitin-proteasome degradation of P-ERK1/2 and P-JNK in aortic valve calcification pathway
PMID: 40225558
6
FHL3 identified as component of cancer prognostic models
PMID: 40855439
7
FHL3 identified as immune cell-specific genetic driver in migraine
PMID: 41029193
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.53Moderate
coronary artery diseaseOpen Targets
0.36Weak
androgenetic alopeciaOpen Targets
0.29Weak
immune system diseaseOpen Targets
0.27Weak
colorectal cancerOpen Targets
0.25Weak
colorectal adenomaOpen Targets
0.17Weak
angina pectorisOpen Targets
0.12Weak
heart diseaseOpen Targets
0.11Weak
neoplasmOpen Targets
0.10Suggestive
gastric cancerOpen Targets
0.08Suggestive
hepatocellular carcinomaOpen Targets
0.08Suggestive
rheumatoid arthritisOpen Targets
0.07Suggestive
Hashimoto's thyroiditisOpen Targets
0.06Suggestive
hypothyroidismOpen Targets
0.06Suggestive
gliomaOpen Targets
0.05Suggestive
melanomaOpen Targets
0.05Suggestive
non-small cell lung carcinomaOpen Targets
0.05Suggestive
myxedemaOpen Targets
0.05Suggestive
Abnormality of the gastrointestinal tractOpen Targets
0.04Suggestive
urinary system diseaseOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
KLF3Protein interaction82%INPP5AProtein interaction79%SMTNShared pathway67%ZBTB42Shared pathway50%SMTNL2Shared pathway50%THSD7AShared pathway50%
Tissue Expression6 tissues
Lung
100%
Ovary
63%
Heart
28%
Brain
20%
Bone Marrow
17%
Liver
16%
Gene Interaction Network
Click a node to explore
FHL3KLF3INPP5ASMTNZBTB42SMTNL2THSD7A
PROTEIN STRUCTURE
Preparing viewer…
PDB1WYH · NMR
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.83LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.58 [0.41–0.83]
RankingsWhere FHL3 stands among ~20K protein-coding genes
  • #5,244of 20,598
    Most Researched91
  • #7,152of 17,882
    Most Constrained (LOEUF)0.83
Genes detectedFHL3
Sources retrieved10 papers
Response time—
📄 Sources
10â–¼
1
Senescent fibroblasts secrete CTHRC1 to promote cancer stemness in hepatocellular carcinoma.
PMID: 40855439
Cell Commun Signal · 2025
1.00
2
Clinical, immunological and genetic findings in patients with UNC13D deficiency (FHL3): A systematic review.
PMID: 32679608
Pediatr Allergy Immunol · 2021
0.90
3
AMBP protects against aortic valve calcification by inhibiting ERK1/2 and JNK pathways mediated by FHL3.
PMID: 40225558
Theranostics · 2025
0.80
4
Prokaryotic expression, purification and functional characterization of human FHL3.
PMID: 19547926
Biotechnol Lett · 2009
0.70
5
FHL3 negatively regulates human high-affinity IgE receptor beta-chain gene expression by acting as a transcriptional co-repressor of MZF-1.
PMID: 15453830
Biochem J · 2005
0.60