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27 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CD99
CD99 molecule (Xg blood group)
Chromosome X|Y · X;Y
NCBI Gene: 4267Ensembl: ENSG00000002586.20HGNC: HGNC:7082UniProt: A0A096LP69
161PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingfocal adhesionT cell extravasationpositive regulation of neutrophil extravasationneurodegenerative diseaseprimary ovarian insufficiencyneoplasmacute myeloid leukemia
✦AI Summary

CD99 is a transmembrane glycoprotein that serves multiple critical functions in immune cell biology and cellular homeostasis. Its primary function involves T-cell adhesion processes, leukocyte extravasation across endothelial barriers, and immunological synapse formation 1. CD99 facilitates leukocyte migration by helping cells overcome the endothelial basement membrane during extravasation, acting independently of PECAM1 1. Mechanistically, CD99 mediates actin-microtubule interactions through its transmembrane and cytoplasmic domains, with the cytoplasmic domain interacting with actin while the transmembrane domain binds microtubules 1. CD99 deficiency abolishes immunological synapse formation and prevents effective T cell immunity 1. Beyond immune function, CD99 regulates cellular differentiation and homeostasis, particularly in keratinocytes where it maintains tissue homeostasis and initiates early differentiation 2. In disease contexts, CD99 is highly expressed in Ewing sarcoma, serving as a diagnostic marker 3, and functions as an immune checkpoint target when PILRα on tumor cells interacts with CD99 on T cells to suppress antitumor immunity 4. Therapeutically, anti-CD99 antibodies show promise in cancer treatment by promoting tumor cell phagocytosis and reprogramming macrophages toward pro-inflammatory phenotypes 5, with potential for combination with immune checkpoint inhibitors 6.

Sources cited
1
Its primary function involves T-cell adhesion processes, leukocyte extravasation across endothelial barriers, and immunological synapse formation .
PMID: 40866332
2
Beyond immune function, CD99 regulates cellular differentiation and homeostasis, particularly in keratinocytes where it maintains tissue homeostasis and initiates early differentiation .
PMID: 35339749
3
In disease contexts, CD99 is highly expressed in Ewing sarcoma, serving as a diagnostic marker , and functions as an immune checkpoint target when PILRα on tumor cells interacts with CD99 on T cells to suppress antitumor immunity .
PMID: 29977059
4
In disease contexts, CD99 is highly expressed in Ewing sarcoma, serving as a diagnostic marker , and functions as an immune checkpoint target when PILRα on tumor cells interacts with CD99 on T cells to suppress antitumor immunity .
PMID: 40312493
5
Therapeutically, anti-CD99 antibodies show promise in cancer treatment by promoting tumor cell phagocytosis and reprogramming macrophages toward pro-inflammatory phenotypes , with potential for combination with immune checkpoint inhibitors .
PMID: 38051221
6
Therapeutically, anti-CD99 antibodies show promise in cancer treatment by promoting tumor cell phagocytosis and reprogramming macrophages toward pro-inflammatory phenotypes , with potential for combination with immune checkpoint inhibitors .
PMID: 40427525
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.25Weak
primary ovarian insufficiencyOpen Targets
0.12Weak
neoplasmOpen Targets
0.11Weak
acute myeloid leukemiaOpen Targets
0.10Suggestive
osteosarcomaOpen Targets
0.09Suggestive
Ewing sarcomaOpen Targets
0.09Suggestive
acute lymphoblastic leukemiaOpen Targets
0.09Suggestive
cancerOpen Targets
0.09Suggestive
gliomaOpen Targets
0.09Suggestive
multiple myelomaOpen Targets
0.08Suggestive
myeloid sarcomaOpen Targets
0.08Suggestive
leukemiaOpen Targets
0.08Suggestive
anaplastic large cell lymphomaOpen Targets
0.07Suggestive
glioblastomaOpen Targets
0.05Suggestive
plasma cell neoplasmOpen Targets
0.04Suggestive
ulcerative colitisOpen Targets
0.04Suggestive
glioblastoma multiformeOpen Targets
0.04Suggestive
focal segmental glomerulosclerosisOpen Targets
0.04Suggestive
ameloblastomaOpen Targets
0.03Suggestive
breast cancerOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PILRBProtein interaction98%SPNProtein interaction98%CD34Protein interaction98%ICAM1Protein interaction98%PILRAProtein interaction97%CD99L2Protein interaction97%
Tissue Expression6 tissues
Ovary
100%
Lung
100%
Liver
74%
Heart
73%
Bone Marrow
22%
Brain
21%
Gene Interaction Network
Click a node to explore
CD99PILRBSPNCD34ICAM1PILRACD99L2
PROTEIN STRUCTURE
Preparing viewer…
PDB7SFX · 3.10 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.98LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.67 [0.47–0.98]
RankingsWhere CD99 stands among ~20K protein-coding genes
  • #2,785of 20,598
    Most Researched161 · top quartile
  • #9,381of 17,882
    Most Constrained (LOEUF)0.98
Genes detectedCD99
Sources retrieved27 papers
Response time—
📄 Sources
27▼
1
Ewing sarcoma.
PMID: 29977059
Nat Rev Dis Primers · 2018
1.00
2
PILRα on tumor cells interacts with the T cell surface protein CD99 to suppress antitumor immunity.
PMID: 40312493
Nat Cancer · 2025
0.90
3
CD99 in malignant hematopoiesis.
PMID: 34920053
Exp Hematol · 2022
0.80
4
Characterization and epitope mapping of two monoclonal antibodies against human CD99.
PMID: 12526082
Exp Mol Med · 2002
0.76
5
Immunoliposome for Ewing Sarcoma.
PMID: 41151854
Cancer Genomics Proteomics · 2025
0.72