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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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TTLL4
tubulin tyrosine ligase like 4
Chromosome 2 · 2q35
NCBI Gene: 9654Ensembl: ENSG00000135912.12HGNC: HGNC:28976UniProt: Q14679
31PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
tubulin bindingprotein-glutamic acid ligase activitytubulin-glutamic acid ligase activitymicrotubule cytoskeleton organizationhepatocellular carcinomaendometriosisOvarian Endometriosisneoplasm
✦AI Summary

TTLL4 (tubulin tyrosine ligase-like 4) is a monoglutamylase that catalyzes the initiation of polyglutamylation, a post-translational modification adding single glutamate residues to target proteins 1. While initially characterized on tubulins—preferentially modifying β-tubulin [UniProt]—TTLL4 has broad substrate specificity including nucleosome assembly proteins (NAP1L1, NAP1L4), the cGAS innate immune sensor, and the pluripotency transcription factor KLF4 2. TTLL4-mediated KLF4 glutamylation protects it from ubiquitin-mediated degradation, promoting cell reprogramming and embryonic development 2. In neural tissue, TTLL4 is a key contributor to polyglutamylation-related neurodegeneration, with TTLL4 deficiency partially rescuing Purkinje cell and photoreceptor loss in polyglutamylation-imbalanced mice 3. TTLL4 activity is negatively regulated by NEK5-mediated phosphorylation 4. Clinically, TTLL4 overexpression in breast cancer associates with brain metastasis by enhancing β-tubulin polyglutamylation and multivesicular body trafficking, promoting blood-brain barrier disruption 5. Similarly, TTLL4 upregulation in pancreatic cancer correlates with PELP1 polyglutamylation and altered chr2 remodeling 6. These findings establish TTLL4 as a regulatory hub linking cytoskeletal dynamics to pluripotency, neuronal homeostasis, and cancer progression.

Sources cited
1
Polyglutamylation adds glutamate residues to proteins; TTLL4 is a major polyglutamylase with broad substrate specificity beyond tubulins
PMID: 18045879
2
TTLL4 deficiency attenuates neurodegeneration in polyglutamylation-imbalanced mice, rescuing Purkinje cell and photoreceptor loss
PMID: 35404950
3
TTLL4 polyglutamylates KLF4 at Glu381, preventing ubiquitination and promoting cell reprogramming and early embryogenesis
PMID: 29593216
4
NEK5 kinase negatively regulates TTLL4 polyglutamylase activity through phosphorylation at Y815 and S1136
PMID: 34084286
5
TTLL4 overexpression in breast cancer increases β-tubulin polyglutamylation and alters exosome trafficking to promote brain metastasis
PMID: 32998758
6
TTLL4 is upregulated in pancreatic cancer and mediates PELP1 polyglutamylation affecting chromatin remodeling
PMID: 20442285
Disease Associationsⓘ20
hepatocellular carcinomaOpen Targets
0.07Suggestive
endometriosisOpen Targets
0.06Suggestive
Ovarian EndometriosisOpen Targets
0.06Suggestive
neoplasmOpen Targets
0.05Suggestive
breast cancerOpen Targets
0.05Suggestive
hypertriglyceridemia 2Open Targets
0.04Suggestive
Familial exudative vitreoretinopathyOpen Targets
0.04Suggestive
Combined hyperlipidemiaOpen Targets
0.04Suggestive
Familial drusenOpen Targets
0.04Suggestive
familial apolipoprotein C-II deficiencyOpen Targets
0.04Suggestive
Hyperlipoproteinemia type 1Open Targets
0.04Suggestive
X-linked retinoschisisOpen Targets
0.03Suggestive
thyroid hormone metabolism, abnormal, 2Open Targets
0.03Suggestive
sitosterolemia 2Open Targets
0.03Suggestive
familial hypercholesterolemiaOpen Targets
0.03Suggestive
homozygous familial hypercholesterolemiaOpen Targets
0.03Suggestive
X-linked retinal dysplasiaOpen Targets
0.03Suggestive
triple-negative breast cancerOpen Targets
0.03Suggestive
male infertilityOpen Targets
0.01Suggestive
infectionOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TTLL11Shared pathway100%TTLL7Shared pathway100%AGBL4Protein interaction81%AGTPBP1Protein interaction78%TTLL12Protein interaction72%TTLL1Shared pathway52%
Tissue Expression6 tissues
Lung
100%
Bone Marrow
98%
Liver
83%
Ovary
66%
Brain
47%
Heart
34%
Gene Interaction Network
Click a node to explore
TTLL4TTLL11TTLL7AGBL4AGTPBP1TTLL12TTLL1
PROTEIN STRUCTURE
Preparing viewer…
PDB5T6X · 1.69 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.75LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.63 [0.52–0.75]
RankingsWhere TTLL4 stands among ~20K protein-coding genes
  • #11,822of 20,598
    Most Researched31
  • #6,005of 17,882
    Most Constrained (LOEUF)0.75
Genes detectedTTLL4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Polyglutamylation is a post-translational modification with a broad range of substrates.
PMID: 18045879
J Biol Chem · 2008
1.00
2
TTLL1 and TTLL4 polyglutamylases are required for the neurodegenerative phenotypes in pcd mice.
PMID: 35404950
PLoS Genet · 2022
0.90
3
Cytosolic carboxypeptidase CCP6 is required for megakaryopoiesis by modulating Mad2 polyglutamylation.
PMID: 25332286
J Exp Med · 2014
0.80
4
Characterization of tubulin post-translational modifications and their enzymes during human oocyte meiosis.
PMID: 39920028
Reprod Biomed Online · 2025
0.70
5
Klf4 glutamylation is required for cell reprogramming and early embryonic development in mice.
PMID: 29593216
Nat Commun · 2018
0.60