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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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DPP9
dipeptidyl peptidase 9
Chromosome 19 Β· 19p13.3
NCBI Gene: 91039Ensembl: ENSG00000142002.19HGNC: HGNC:18648UniProt: M0R2A8
90PubMed Papers
21Diseases
0Drugs
4Pathogenic Variants
FUNCTIONAL ROLE
Protease
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
pyroptotic inflammatory responseprotein bindingnegative regulation of programmed cell deathcytosolhatipoglu immunodeficiency syndromeCOVID-19idiopathic pulmonary fibrosisneurodegenerative disease
✦AI Summary

DPP9 is a dipeptidyl peptidase that cleaves N-terminal dipeptides from proteins with Pro or Ala at position 2 1. Its primary function is inhibiting caspase-1-dependent pyroptosis by suppressing NLRP1 and CARD8 inflammasome activation 23. DPP9 sequesters cleaved C-terminal fragments of NLRP1 and CARD8 in ternary complexes, preventing their oligomerization 4. Interestingly, this inhibitory activity requires DPP9's dipeptidyl peptidase activity, though NLRP1 and CARD8 are not direct substrates 4. Beyond inflammasome regulation, DPP9 has emerged as a key regulator of cellular stress responses and cancer biology. DPP9 stabilizes NRF2 by competing with NRF2 for KEAP1 binding in an enzyme-independent manner, suppressing ferroptosis and promoting sorafenib resistance in renal cell carcinoma 5. Similarly, DPP9 regulates NQO1 and ROS levels to enhance chemotherapy resistance in liver cancer 6. Additional substrates include Syk, AK2, and BRCA2, whose N-terminal processing triggers proteasomal degradation 7. Clinically, DPP9 dysfunction causes Hatipoglu immunodeficiency syndrome, with DPP9 deficiency resulting in excessive NLRP1-driven inflammation 8. Genetic variants in DPP9 associate with severe COVID-19, implicating it in antiviral defense 9.

Sources cited
1
DPP9 cleaves N-terminal dipeptides from proteins with Pro or Ala at position 2
PMID: 12662155
2
DPP9 inhibits caspase-1-dependent pyroptosis by suppressing NLRP1 and CARD8
PMID: 27820798
3
DPP9 represses NLRP1 inflammasome through both peptidase activity and FIIND domain binding
PMID: 30291141
4
DPP9 sequesters cleaved C-terminal NLRP1/CARD8 fragments in ternary complexes; requires peptidase activity but these are not bona fide substrates
PMID: 33731929
5
DPP9 stabilizes NRF2 by competing with NRF2 for KEAP1 binding, suppressing ferroptosis and inducing sorafenib resistance in renal cell carcinoma
PMID: 37713596
6
DPP9 regulates NQO1 and ROS levels through NRF2/KEAP1 pathway to promote chemotherapy resistance in liver cancer
PMID: 39094401
7
DPP9 processes substrates Syk, AK2, and BRCA2 via N-terminal cleavage triggering proteasomal degradation
PMID: 37230592
8
DPP9 deficiency causes immunodeficiency with excessive NLRP1-driven inflammation; Mendelian disorder rescued by reducing NLRP1/IL-1 signaling
PMID: 36112693
9
DPP9 genetic variants associate with critical illness in COVID-19, implicating role in antiviral defense
PMID: 33307546
Disease Associationsβ“˜21
hatipoglu immunodeficiency syndromeOpen Targets
0.60Moderate
COVID-19Open Targets
0.52Moderate
idiopathic pulmonary fibrosisOpen Targets
0.42Moderate
neurodegenerative diseaseOpen Targets
0.40Weak
interstitial lung diseaseOpen Targets
0.31Weak
respiratory failureOpen Targets
0.27Weak
osteoarthritisOpen Targets
0.27Weak
Abnormality of the gastrointestinal tractOpen Targets
0.22Weak
hepatocellular carcinomaOpen Targets
0.09Suggestive
liver cancerOpen Targets
0.08Suggestive
non-small cell lung carcinomaOpen Targets
0.08Suggestive
oral squamous cell carcinomaOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.06Suggestive
essential tremorOpen Targets
0.06Suggestive
acute kidney injuryOpen Targets
0.06Suggestive
Young adult-onset ParkinsonismOpen Targets
0.06Suggestive
dystonia 27Open Targets
0.05Suggestive
Cranio-cervical dystonia with laryngeal and upper-limb involvementOpen Targets
0.05Suggestive
familial primary pulmonary hypoplasiaOpen Targets
0.05Suggestive
Primary dystonia, DYT13 typeOpen Targets
0.05Suggestive
Hatipoglu immunodeficiency syndromeUniProt
Pathogenic Variants4
NM_139159.5(DPP9):c.2482C>T (p.Arg828Cys)Likely pathogenic
Hatipoglu immunodeficiency syndrome
β˜…β˜†β˜†β˜†2025β†’ Residue 828
NM_139159.5(DPP9):c.2178G>A (p.Met726Ile)Likely pathogenic
Susceptibility to severe COVID-19
β˜…β˜†β˜†β˜†2024β†’ Residue 726
NM_139159.5(DPP9):c.2551C>T (p.Gln851Ter)Pathogenic
Hatipoglu immunodeficiency syndrome
β˜†β˜†β˜†β˜†2023β†’ Residue 851
NM_139159.5(DPP9):c.331C>T (p.Arg111Ter)Pathogenic
Hatipoglu immunodeficiency syndrome
β˜†β˜†β˜†β˜†2023β†’ Residue 111
View on ClinVar β†—
Related Genes
CARD8Protein interaction97%NLRP1Protein interaction95%PREPProtein interaction95%DPP7Protein interaction88%TFDP3Protein interaction79%PEPDShared pathway50%
Tissue Expression6 tissues
Liver
100%
Lung
70%
Heart
41%
Ovary
37%
Bone Marrow
34%
Brain
20%
Gene Interaction Network
Click a node to explore
DPP9CARD8NLRP1PREPDPP7TFDP3PEPD
PROTEIN STRUCTURE
Preparing viewer…
PDB7ZXS Β· 1.81 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.65LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.51 [0.41–0.65]
RankingsWhere DPP9 stands among ~20K protein-coding genes
  • #5,302of 20,598
    Most Researched90
  • #3,755of 5,498
    Most Pathogenic Variants4
  • #4,668of 17,882
    Most Constrained (LOEUF)0.65
Genes detectedDPP9
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
DPP9 Stabilizes NRF2 to Suppress Ferroptosis and Induce Sorafenib Resistance in Clear Cell Renal Cell Carcinoma.
PMID: 37713596
Cancer Res Β· 2023
1.00
2
P38 kinases mediate NLRP1 inflammasome activation after ribotoxic stress response and virus infection.
PMID: 36315050
J Exp Med Β· 2023
0.90
3
Multimodal cell maps as a foundation for structural and functional genomics.
PMID: 40205054
Nature Β· 2025
0.80
4
DPP9 deficiency: An inflammasomopathy that can be rescued by lowering NLRP1/IL-1 signaling.
PMID: 36112693
Sci Immunol Β· 2022
0.70
5
Genetic mechanisms of critical illness in COVID-19.
PMID: 33307546
Nature Β· 2021
0.60