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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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AGBL4
AGBL carboxypeptidase 4
Chromosome 1 · 1p33
NCBI Gene: 84871Ensembl: ENSG00000186094.18HGNC: HGNC:25892UniProt: Q5VU57
17PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
Golgi apparatuscentrioleciliary basal bodycytoplasmatrial fibrillationobesityAbnormality of the skeletal systemmajor depressive disorder
✦AI Summary

AGBL4 is a metallocarboxypeptidase that catalyzes protein deglutamylation, primarily removing polyglutamate side chains from tubulin and non-tubulin proteins 1. The enzyme specifically cleaves tubulin long-side-chains while preserving the branching point glutamate, and also deglutamylates non-tubulin substrates including MYLK and KLF4 1. AGBL4-mediated KLF4 deglutamylation promotes proteasome-mediated degradation, negatively regulating cell pluripotency and embryogenesis 1. Beyond tubulin modification, AGBL4 plays roles in neuronal differentiation 2 and immune responses. In disease contexts, AGBL4 upregulation promotes glioblastoma malignant progression through modulation of MMP-1 and inflammatory pathways; AGBL4 knockdown inhibits GBM cell proliferation, migration, and invasion 3. AGBL4 expression differences distinguish non-progressive from progressive sarcoidosis in bronchoalveolar lavage cells, suggesting involvement in disease progression mechanisms 4. Additionally, AGBL4 genetic variants associate with cardiometabolic risk, including lipid metabolism alterations in population-specific haplotypes 5. AGBL4 polymorphisms correlate with susceptibility to antituberculosis drug-induced hepatotoxicity, with population-specific genotype frequencies affecting DILI risk in Peruvian subpopulations 67. Clinically, AGBL4 represents both a potential biomarker and therapeutic target for managing cancer progression, inflammatory diseases, and adverse drug reactions.

Sources cited
1
AGBL4 catalyzes tubulin deglutamylation and KLF4 deglutamylation affecting cell pluripotency and embryogenesis
PMID: 29593216
2
AGBL4 involved in neuronal differentiation markers in neuroprotection studies
PMID: 38287296
3
AGBL4 upregulation promotes glioblastoma progression via MMP-1 and inflammatory pathway modulation
PMID: 39007144
4
AGBL4 expression differences associate with sarcoidosis disease progression
PMID: 39080656
5
AGBL4 genetic variants associate with cardiometabolic risk and lipid metabolism
PMID: 27853278
6
AGBL4 polymorphisms associate with antituberculosis drug-induced hepatotoxicity susceptibility
PMID: 30720667
7
AGBL4 genotype frequencies vary among Peruvian populations affecting drug-induced liver injury risk
PMID: 40517315
Disease Associationsⓘ20
atrial fibrillationOpen Targets
0.46Moderate
obesityOpen Targets
0.42Moderate
Abnormality of the skeletal systemOpen Targets
0.39Weak
major depressive disorderOpen Targets
0.37Weak
smoking initiationOpen Targets
0.36Weak
overnutritionOpen Targets
0.35Weak
liver diseaseOpen Targets
0.34Weak
poisoningOpen Targets
0.34Weak
response to xenobiotic stimulusOpen Targets
0.31Weak
male reproductive organ cancerOpen Targets
0.28Weak
ovarian dysfunctionOpen Targets
0.28Weak
hyperpituitarismOpen Targets
0.28Weak
schizophreniaOpen Targets
0.27Weak
Abruptio PlacentaeOpen Targets
0.27Weak
eye diseaseOpen Targets
0.27Weak
head injuryOpen Targets
0.27Weak
cervical carcinomaOpen Targets
0.26Weak
pneumococcal pneumoniaOpen Targets
0.26Weak
multinodular goiterOpen Targets
0.25Weak
spermatoceleOpen Targets
0.25Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
AGBL5Shared pathway100%BEND5Protein interaction87%TTLL4Protein interaction81%TTLL6Protein interaction74%PGA4Shared pathway50%PGA3Shared pathway50%
Tissue Expression6 tissues
Brain
100%
Liver
17%
Bone Marrow
10%
Heart
10%
Ovary
5%
Lung
2%
Gene Interaction Network
Click a node to explore
AGBL4AGBL5BEND5TTLL4TTLL6PGA4PGA3
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q5VU57
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.11LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.87 [0.69–1.11]
RankingsWhere AGBL4 stands among ~20K protein-coding genes
  • #14,917of 20,598
    Most Researched17
  • #11,336of 17,882
    Most Constrained (LOEUF)1.11
Genes detectedAGBL4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Tirzepatide prevents neurodegeneration through multiple molecular pathways.
PMID: 38287296
J Transl Med · 2024
1.00
2
AGBL4 promotes malignant progression of glioblastoma via modulation of MMP-1 and inflammatory pathways.
PMID: 39007144
Front Immunol · 2024
0.90
3
Multi-omic signatures of sarcoidosis and progression in bronchoalveolar lavage cells.
PMID: 39080656
Respir Res · 2024
0.80
4
Integrative mutation, haplotype and G × G interaction evidence connects ABGL4, LRP8 and PCSK9 genes to cardiometabolic risk.
PMID: 27853278
Sci Rep · 2016
0.70
5
Discovery of First-In-Class Potent and Selective Tropomyosin Receptor Kinase Degraders.
PMID: 33058680
J Med Chem · 2020
0.60