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25 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CBLB
Cbl proto-oncogene B
Chromosome 3 · 3q13.11
NCBI Gene: 868Ensembl: ENSG00000114423.24HGNC: HGNC:1542UniProt: B3KSS7
206PubMed Papers
21Diseases
0Drugs
3Pathogenic Variants
RESEARCH IMPACT
Trending
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein stabilizationnegative regulation of T cell activationprotein K63-linked ubiquitinationplasma membraneautoimmune diseasehypothyroidismurticariamyxedema
✦AI Summary

CBLB (Cbl proto-oncogene B) is an E3 ubiquitin ligase that functions as a master negative regulator of T cell and B cell activation 1. The protein catalyzes Lys-63-linked ubiquitination of multiple substrates including PIK3R1/p85, VAV1, and PLCG1, thereby suppressing T cell receptor (TCR) signaling and imposing a requirement for CD28 costimulation for T cell proliferation 2. In LAG3-mediated immune checkpoint inhibition, CBLB ubiquitinates LAG3 upon ligand engagement, unleashing its inhibitory signaling function 2. CBLB also regulates B cell responses by promoting SYK ubiquitination and degradation 2. Clinically, CBLB dysregulation contributes to autoimmune disease pathogenesis. Deficiency of CBL and CBLB in CD4+ T cells causes hyper T follicular helper cell responses and systemic lupus erythematosus through impaired BCL6 degradation 3. Conversely, CBLB inhibition presents therapeutic opportunities in cancer immunotherapy. CBLB is upregulated in exhausted CD8+ tumor-infiltrating lymphocytes, and its deletion restores effector function and prevents CAR T cell exhaustion 4. Pharmacological CBLB inhibition via NX-1607 enhances T cell activation through MAPK/ERK pathway signaling and demonstrates antitumor activity in murine lymphoma models 5. These findings position CBLB as a promising immunotherapeutic target for both enhancing anti-tumor immunity and managing immune dysregulation.

Sources cited
1
CBLB is a master regulator of CD28 and CTLA-4 signaling that promotes immunosuppression in tumor microenvironment
PMID: 36750253
2
CBLB mediates Lys-63-linked ubiquitination of LAG3 upon ligand engagement to promote its inhibitory function and suppress antitumor immunity
PMID: 40101708
3
CBL and CBLB deficiency in CD4+ T cells causes hyper Tfh cell responses and SLE through impaired BCL6 degradation via ICOS ubiquitination
PMID: 38761804
4
CBLB is upregulated in exhausted CD8+ TILs; its deletion restores effector function and prevents CAR T cell exhaustion
PMID: 33462140
5
CBLB inhibitor NX-1607 enhances T cell activation through PLCγ1 and MAPK/ERK signaling and reduces tumor growth in lymphoma models
PMID: 40447319
Disease Associationsⓘ21
autoimmune diseaseOpen Targets
0.58Moderate
hypothyroidismOpen Targets
0.48Moderate
urticariaOpen Targets
0.44Moderate
myxedemaOpen Targets
0.43Moderate
multiple sclerosisOpen Targets
0.41Moderate
esophageal adenocarcinomaOpen Targets
0.38Weak
pancreatic ductal adenocarcinomaOpen Targets
0.37Weak
thyroid diseaseOpen Targets
0.37Weak
lung carcinomaOpen Targets
0.37Weak
bile duct carcinomaOpen Targets
0.37Weak
breast ductal adenocarcinomaOpen Targets
0.37Weak
carcinoma of liver and intrahepatic biliary tractOpen Targets
0.37Weak
cutaneous melanomaOpen Targets
0.37Weak
Hepatobiliary NeoplasmOpen Targets
0.37Weak
kidney neoplasmOpen Targets
0.37Weak
lymphoid neoplasmOpen Targets
0.37Weak
nodular melanomaOpen Targets
0.37Weak
prostate adenocarcinomaOpen Targets
0.37Weak
superficial spreading melanomaOpen Targets
0.37Weak
Uterine CarcinosarcomaOpen Targets
0.37Weak
Autoimmune disease, multisystem, infantile-onset, 3UniProt
Pathogenic Variants3
NM_170662.5(CBLB):c.770A>T (p.His257Leu)Pathogenic
Autoimmune disease, multisystem, infantile-onset, 3
☆☆☆☆2023→ Residue 257
NM_170662.5(CBLB):c.1402C>T (p.Arg468Ter)Pathogenic
Autoimmune disease, multisystem, infantile-onset, 3
☆☆☆☆2023→ Residue 468
NM_170662.5(CBLB):c.1308C>G (p.Cys436Trp)Pathogenic
Autoimmune disease, multisystem, infantile-onset, 3
☆☆☆☆2023→ Residue 436
View on ClinVar ↗
Related Genes
PIK3R3Protein interaction100%PIK3R1Protein interaction100%PIK3CBProtein interaction100%PIK3CDProtein interaction100%PIK3CAProtein interaction100%RAPGEF1Protein interaction100%
Tissue Expression6 tissues
Lung
100%
Heart
95%
Ovary
92%
Bone Marrow
49%
Brain
42%
Liver
41%
Gene Interaction Network
Click a node to explore
CBLBPIK3R3PIK3R1PIK3CBPIK3CDPIK3CARAPGEF1
PROTEIN STRUCTURE
Preparing viewer…
PDB8QTG · 1.42 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.41Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.28 [0.20–0.41]
RankingsWhere CBLB stands among ~20K protein-coding genes
  • #2,023of 20,598
    Most Researched206 · top 10%
  • #3,933of 5,498
    Most Pathogenic Variants3
  • #2,063of 17,882
    Most Constrained (LOEUF)0.41 · top quartile
Genes detectedCBLB
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
Targeting Cbl-b in cancer immunotherapy.
PMID: 36750253
J Immunother Cancer · 2023
1.00
2
Ligand-induced ubiquitination unleashes LAG3 immune checkpoint function by hindering membrane sequestration of signaling motifs.
PMID: 40101708
Cell · 2025
0.90
3
Deficiency of CBL and CBLB ubiquitin ligases leads to hyper T follicular helper cell responses and lupus by reducing BCL6 degradation.
PMID: 38761804
Immunity · 2024
0.80
4
Deletion of Cbl-b inhibits CD8
PMID: 33462140
J Immunother Cancer · 2021
0.70
5
PD-1/LAG-3 co-signaling profiling uncovers CBL ubiquitin ligases as key immunotherapy targets.
PMID: 39030301
EMBO Mol Med · 2024
0.64