HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
TTLL11
tubulin tyrosine ligase like 11
Chromosome 9 · 9q33.2
NCBI Gene: 158135Ensembl: ENSG00000175764.18HGNC: HGNC:18113UniProt: Q6ZUZ3
18PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
microtubule cytoskeleton organizationtubulin-glutamic acid ligase activityciliary basal bodytubulin bindingosteoarthritisalcohol drinkingintelligenceamyloidosis
✦AI Summary

TTLL11 is a polyglutamylase enzyme that catalyzes ATP-dependent polyglutamylation of tubulin proteins, preferentially elongating polyglutamate chains rather than initiating them 1. The enzyme exhibits a unique bipartite microtubule recognition strategy, engaging adjacent protofilaments to extend primary polypeptide chains of both α- and β-tubulin 1. Beyond tubulin modification, TTLL11 also polyglutamylates non-tubulin substrates like Dishevelled 3 (DVL3) through carboxy-terminal polyglutamylation, affecting protein phase separation and Wnt signaling 2. TTLL11 is essential for proper chromosome 9 during mitosis, with reduced levels compromising segregation fidelity and causing chromosome 9 3. The enzyme plays critical roles in neuronal development by regulating α-tubulin polyglutamylation, which restricts neurite branching when working synergistically with doublecortin 4. Clinically, TTLL11 mutations are associated with idiopathic scoliosis through ciliary defects 5, and genetic variants correlate with sustained attention performance and ADHD susceptibility 6. The gene is systematically downregulated in cancer cells, linking reduced TTLL11 expression to aneuploidy and chr9 instability 3. TTLL11 is also involved in oocyte meiosis and drug response variability in Kawasaki disease 78.

Sources cited
1
TTLL11 is a polyglutamylase with unique bipartite MT recognition strategy that extends primary tubulin chains
PMID: 40834096
2
TTLL11 performs carboxy-terminal polyglutamylation of DVL3, affecting phase separation and Wnt signaling
PMID: 39349846
3
TTLL11 is essential for chromosome segregation fidelity and is downregulated in cancer cells
PMID: 36414642
4
TTLL11 works synergistically with doublecortin to regulate α-tubulin polyglutamylation and restrict neurite branching
PMID: 39966472
5
TTLL11 mutations cause ciliary defects associated with idiopathic scoliosis
PMID: 34040021
6
TTLL11 genetic variants are associated with sustained attention performance and ADHD
PMID: 36511133
7
TTLL11 is expressed during human oocyte meiosis
PMID: 39920028
8
TTLL11 variants are associated with drug response variability in Kawasaki disease
PMID: 38259468
Disease Associationsⓘ20
osteoarthritisOpen Targets
0.37Weak
alcohol drinkingOpen Targets
0.33Weak
intelligenceOpen Targets
0.30Weak
amyloidosisOpen Targets
0.30Weak
HypocalcemiaOpen Targets
0.28Weak
corneal neovascularizationOpen Targets
0.28Weak
obesityOpen Targets
0.27Weak
mathematical abilityOpen Targets
0.27Weak
response to antihypertensive drugOpen Targets
0.26Weak
chondrocalcinosisOpen Targets
0.25Weak
hypopituitarismOpen Targets
0.25Weak
schizophreniaOpen Targets
0.23Weak
sign or symptomOpen Targets
0.10Weak
cancerOpen Targets
0.06Suggestive
neoplasmOpen Targets
0.05Suggestive
injuryOpen Targets
0.03Suggestive
hereditary spastic paraplegiaOpen Targets
0.01Suggestive
idiopathic scoliosisOpen Targets
0.01Suggestive
Åland Islands eye diseaseOpen Targets
0.01Suggestive
scoliosisOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TTLL4Shared pathway100%TTLL7Shared pathway100%TTLL1Shared pathway67%TXNDC9Shared pathway50%MAP7D1Shared pathway50%CGNShared pathway50%
Tissue Expression6 tissues
Brain
100%
Heart
85%
Ovary
62%
Liver
59%
Lung
51%
Bone Marrow
35%
Gene Interaction Network
Click a node to explore
TTLL11TTLL4TTLL7TTLL1TXNDC9MAP7D1CGN
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q6ZUZ3
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.04LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.78 [0.58–1.04]
RankingsWhere TTLL11 stands among ~20K protein-coding genes
  • #14,858of 20,598
    Most Researched18
  • #10,420of 17,882
    Most Constrained (LOEUF)1.04
Genes detectedTTLL11
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Carboxy-terminal polyglutamylation regulates signaling and phase separation of the Dishevelled protein.
PMID: 39349846
EMBO J · 2024
1.00
2
Doublecortin restricts neuronal branching by regulating tubulin polyglutamylation.
PMID: 39966472
Nat Commun · 2025
0.90
3
TTLL11 gene is associated with sustained attention performance and brain networks: A genome-wide association study of a healthy Chinese sample.
PMID: 36511133
Genes Brain Behav · 2023
0.80
4
Genetic variant of TTLL11 gene and subsequent ciliary defects are associated with idiopathic scoliosis in a 5-generation UK family.
PMID: 34040021
Sci Rep · 2021
0.70
5
Mechanistic insights into TTLL11 polyglutamylase-mediated primary tubulin chain elongation.
PMID: 40834096
Sci Adv · 2025
0.60