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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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UNC119B
unc-119 lipid binding chaperone B
Chromosome 12 · 12q24.31
NCBI Gene: 84747Ensembl: ENSG00000175970.13HGNC: HGNC:16488UniProt: A6NIH7
39PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
sperm end piecelipid bindingsperm midpiecesperm principal pieceneurodegenerative diseasesubstance-related disorderhepatocellular carcinomaInsulin resistance
✦AI Summary

UNC119B is a myristoyl-binding chaperone that facilitates trafficking of N-terminally myristoylated proteins to specific cellular compartments 1. It functions as a lipid-binding adapter with an immunoglobulin-like fold that masks hydrophobic myristoyl moieties, enabling transport through the hydrophilic cytosol 1. UNC119B demonstrates selective cargo binding, with affinity variations up to 427-fold for different myristoylated substrates 2. A key biological role involves localizing myristoylated NPHP3 to primary cilia; UNC119B knockdown prevents this ciliary trafficking 1. The protein is released from cargo through interaction with small GTPases ARL2 and ARL3 in a GTP-dependent manner 3. Beyond cilia biology, UNC119B negatively regulates glucose homeostasis by inhibiting Rac1 activation, thereby impairing GLUT4 translocation in response to insulin 4. UNC119B overexpression correlates with poor prognosis in hepatocellular carcinoma, contributing to immunotherapy resistance and chemotherapy resistance 5. Additionally, UNC119B serves as a protein interactor that allosterically activates PSKH1 kinase 6. Genetic compensation mechanisms upregulate UNC119B in CEP290-deficient models to partially rescue ciliary defects 7.

Sources cited
1
UNC119B functions as a myristoyl-binding chaperone for N-myristoylated proteins and facilitates NPHP3 trafficking to cilia
PMID: 23000199
2
UNC119B has a hydrophobic pocket binding myristoyl lipids and shows 427-fold variation in binding affinities for different cargo peptides
PMID: 34130453
3
UNC119B shuttles lipid-modified proteins to cilia with cargo release mediated by ARL3 in GTP-dependent manner
PMID: 27911709
4
UNC119B inhibits Rac1 activation and GLUT4 translocation, regulating insulin sensitivity and glucose homeostasis
PMID: 40754229
5
UNC119B overexpression in hepatocellular carcinoma associates with poor prognosis, immunotherapy resistance, and chemotherapy resistance
PMID: 37903971
6
UNC119B is a protein interactor that activates PSKH1 kinase via allosteric mechanisms
PMID: 39964718
7
UNC119B upregulation provides genetic compensation to rescue ciliary defects in CEP290 mutants
PMID: 34155518
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.43Moderate
substance-related disorderOpen Targets
0.12Weak
hepatocellular carcinomaOpen Targets
0.08Suggestive
Insulin resistanceOpen Targets
0.05Suggestive
type 2 diabetes mellitusOpen Targets
0.04Suggestive
diabetes mellitusOpen Targets
0.02Suggestive
corneal degenerationOpen Targets
0.02Suggestive
bipolar disorderOpen Targets
0.02Suggestive
major depressive disorderOpen Targets
0.02Suggestive
neoplasmOpen Targets
0.01Suggestive
obesityOpen Targets
0.01Suggestive
ciliopathyOpen Targets
0.01Suggestive
Joubert syndromeOpen Targets
0.00Suggestive
ulcerative colitisOpen Targets
0.00Suggestive
nephronophthisisOpen Targets
0.00Suggestive
cardiovascular diseaseOpen Targets
0.00Suggestive
diffuse large B-cell lymphomaOpen Targets
0.00Suggestive
Hereditary breast cancerOpen Targets
0.00Suggestive
hereditary breast carcinomaOpen Targets
0.00Suggestive
uterine corpus endometrial carcinomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PDE6DProtein interaction100%NPHP3Protein interaction100%ARL3Protein interaction93%RP2Protein interaction93%CYS1Protein interaction88%PSKH2Protein interaction81%
Tissue Expression6 tissues
Ovary
100%
Brain
67%
Bone Marrow
66%
Heart
53%
Lung
49%
Liver
40%
Gene Interaction Network
Click a node to explore
UNC119BPDE6DNPHP3ARL3RP2CYS1PSKH2
PROTEIN STRUCTURE
Preparing viewer…
PDB7OK6 · 1.95 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.17LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.75 [0.49–1.17]
RankingsWhere UNC119B stands among ~20K protein-coding genes
  • #10,414of 20,598
    Most Researched39
  • #12,265of 17,882
    Most Constrained (LOEUF)1.17
Genes detectedUNC119B
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Integrated analysis of histone lysine lactylation (Kla)-specific genes suggests that NR6A1, OSBP2 and UNC119B are novel therapeutic targets for hepatocellular carcinoma.
PMID: 37903971
Sci Rep · 2023
1.00
2
The Structural and Biochemical Characterization of UNC119B Cargo Binding and Release Mechanisms.
PMID: 34130453
Biochemistry · 2021
0.90
3
Shuttling and sorting lipid-modified cargo into the cilia.
PMID: 27911709
Biochem Soc Trans · 2016
0.80
4
Inhibition of Unc119b improves insulin sensitivity through potentiation of Rac1 activation in skeletal muscle and brown adipose tissue.
PMID: 40754229
Mol Metab · 2025
0.70
5
Genetic compensation for cilia defects in cep290 mutants by upregulation of cilia-associated small GTPases.
PMID: 34155518
J Cell Sci · 2021
0.60