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. 2021 Feb 15;42(11):1742–1756. doi: 10.1038/s41401-020-00603-2

Table 2.

Novel experimental agents targeting NLRs for autoimmune diseases treatment

Disease Targets Agents Experimental model Outcomes Potential mechanisms References
IBD NLRC2 SB203580 DSS-induced murine colitis ↓Clinical score, ↓Inflammatory cytokines Blocking activation of p38/MAPK and RIP2/NOD2/NF-κB signaling pathways [105]
GSK583 Intestinal mucosal tissue from IBD patients ↓Inflammatory cytokines Blocking activation of RIP2/NOD2 signaling pathway [106]
NLRP3 Wogonoside THP-1 cell, DSS-induced murine colitis ↓Inflammatory cells infiltration Blocking activation of NF-κB and NLRP3 inflammasome [111]
Alpinetin THP-1 cell, DSS-induced murine colitis ↓Colonic pathological damage Suppressing TLR4 and NLRP3 signaling pathways [112]
Oroxylin A THP-1 cell, BMDM, DSS-induced murine colitis ↓Inflammatory cells infiltration Suppressing ASC speck formation and NLRP3 inflammasome assembly, inhibiting NF-κB p65 expression and nuclear translocation [113]
Naringin RAW264.7 cell, DSS-induced murine colitis ↓DAI, ↓colonic pathological damage Blocking activation of NF-κB, MAPK and NLRP3 inflammasome [114]
Asiatic acid THP-1 cell, DSS-induced murine colitis ↓DAI, ↓histopathologic scores Suppressing mitochondria-mediated NLRP3 inflammasome activation [115]
Dimethyl fumarate THP-1 cell, BMDM, DSS-induced murine colitis ↓Colonic pathological damage Activating Nrf2 and suppressing NLRP3 inflammasome activation [116]
miR-223 BMDM, neutrophil, DSS-induced murine colitis ↓Inflammatory cytokines Controlling myeloid-specific NLRP3 inflammasome activity [117]
MCC950 Spontaneous chronic colitis mouse model Winnie ↓DAI, ↓colonic pathological damage Small-molecule inhibitor of NLRP3, blocking activation of NLRP3 inflammasome [118]
NLRP1 SDG RAW264.7 cell, DSS-induced murine colitis ↓Macrophage infiltration, ↓histopathologic scores Blocking activation of NLRP1 inflammasome and NF-κB [119]
NLRP6 Apigenin DSS-induced murine colitis ↓DAI, ↓colonic pathological damage Modulating gut microbiota through regulating NLRP6 signaling pathway [120]
CIITA Curcumin Colonic epithelial cells ↓Disease severity, ↓T cell chemokines Suppressing transcription level of MHC-II and CIITA induced by IFN-γ [149]
NLRX1 NX-13 DSS-induced murine colitis ↓Disease severity, ↓leukocytic infiltration Activating NLRX1 to mediate a resistance to both NF-κB activity and oxidative phosphorylation [151]
RA NLRC2 2E7 Collagen-induced arthritis murine model, Collagen antibody-induced arthritis murine model ↓Paw Swelling, ↓inflammatory cytokines Targeting MDP and blocking NOD2-mediated pathways [107]
NLRC5 FER1L4 CFA-induced adjuvant arthritis rat model ↓Paw Swelling, ↓inflammatory cytokines Targeting NLRC5 and decreasing expression of NLRC5 [109]
MEG3 CFA-induced adjuvant arthritis rat model ↓Paw Swelling, ↓inflammatory cytokines Targeting NLRC5 and decreasing expression of NLRC5 [110]
NLRP3 Taraxasterol IL-1β-stimulated human FLS, Collagen-induced arthritis murine model ↓Paw Swelling, ↓inflammatory cytokines Blocking activation of NF-κB and suppressing NLRP3 inflammasome through blocking expressions of NLRP3, ASC and caspase-1 [121]
Curculigoside A CFA-induced adjuvant arthritis rat model ↓Arthritis index, ↓inflammatory cytokines Blocking activation of NF-κB and NLRP3 inflammasome [122]
Methotrexate Collagen-induced arthritis murine model ↓Paw Swelling, ↓inflammatory cytokines Blocking the activation of NF-κB and NLRP3/Caspase-1 pathways, regulating the inflammation related metabolic networks [123]
SLE NLRP3 Icariin MRL/lpr mice ↓Renal tissue damage, ↓inflammatory cytokines Blocking activation of NF-κB and NLRP3 inflammasome [125]
Sophocarpine MRL/lpr mice ↓Urine protein, ↓renal tissue damage Blocking activation of NF-κB and NLRP3 inflammasome [126]
Piperine HK-2 cell, Pristane-induced SLE murine model ↓Inflammatory cytokines Blocking AMPK-mediated activation of NLRP3 inflammasome [127]
Curcumin MRL/lpr mice ↓Urine protein, ↓renal tissue damage Blocking activation of NLRP3 inflammasome [128]
Procyanidin B2 MRL/lpr mice ↓Urine protein, ↓renal tissue damage Inhibiting the activation of NLRP3 inflammasome and the production of cytokines [129]
Melatonin Pristane-induced SLE murine model ↓Renal tissue damage Enhancing the Nrf2 activation and inhibiting NLRP3 inflammasome activation [130]
Baicalein MDSC, Pristane-induced murine model ↓Urine protein, ↓renal tissue damage Regulating the balance of the Nrf2/HO-1 signal and NLRP3 expression [131]
M1 MDSC, J774A.1 cell, LPS-induced murine ASLN model ↓Urine protein, ↓renal tissue damage Activating the sirtuin3/autophagy axis and inhibiting NLRP3 inflammasome activation [133]
Let-7f-5p SLE patients, MRL/lpr mice ↓Inflammatory cytokines Directly targeting NLRP3 and inhibiting NLRP3 expression [134]
Psoriasis NLRP3 BAY 11-7082 IMQ-induced psoriasis-like dermatitis murine model ↓Epidermal thickness, ↓inflammatory cells infiltration Blocking activation of NF-κB and NLRP3 inflammasome [135]
Cycloastragenol BMDM, IMQ-induced psoriasis-like dermatitis murine model ↓epidermal thickness, ↓dermal infiltration of macrophages Suppressing NLRP3 inflammasome complex assembly and NLRP3 inflammasome-mediated pyroptosis [138]
Datura Metel L. IMQ-induced psoriasis-like dermatitis murine model ↓Inflammatory cytokines Decreasing expression of IKKα, NF-κB, ASC, NLRP3 and caspase-1 [137]
MS NLRC2 2E7 MOG35–55-induced EAE murine model ↓Progress and ↓severity Targeting MDP and blocking NOD2-mediated pathways [107]
MIS416 MOG35–55-induced EAE murine model ↓Progress and ↓severity Inhibiting inflammatory T-cell activity by activating TLR9 and NOD2 [108]
NLRP3 MCC950 BMDM, MOG35–55-induced EAE murine model ↓Clinical symptoms, ↓inflammatory cytokines Small-molecule inhibitor of NLRP3, blocking activation of NLRP3 inflammasome [139]
JC-171 BMDM, J774A.1 cell, MOG35–55-induced EAE murine model ↓Progress and ↓severity Small-molecule inhibitor of NLRP3 inflammasome, inhibiting NLRP3/ASC interaction and IL-1β production [141]
OLT1177 MOG35–55-induced EAE murine model ↓Infiltration of CD4 T cells and macrophages Selective inhibitor of NLRP3 inflammasome, inhibiting production of inflammatory cytokines [142]
Prednisone Cuprizone-induced demyelination murine model ↓Progress and ↓severity Blocking activation of NLRP3 inflammasome and production of related inflammatory cytokines [143]
Tetramethylpyrazine MOG35–55-induced EAE murine model ↓Clinical scores and ↓demyelination Reducing the expression of NLRP3 inflammasome and modulating the Th1/Th17/Th2 response [144]
CIITA Diarylpropionitrile PLP-induced EAE murine model ↓Progress and ↓severity Abrogating CIITA expression and hampering IRF-1 to translocate into nucleus [150]
T1D NLRP3 LMP Non-obese diabetic mice ↓Progress and ↓fasting glucose levels Upregulating claudin-1 and ZO-2 expression, blocking NLRP3 inflammasome activation, increasing Treg frequencies [146]
Ginsenoside Rg1 Streptozotocin-induced T1D murine model ↓Fasting glucose levels, ↑insulin secretion Blocking activation of NLRP3 and regulating Keap1/Nrf2/HO-1 pathways [147]
AIH NLRP3 miR223 Hepatic S100-induced AIH murine model ↓Liver injury Downregulating the expression of NLRP3 and caspase-1 [148]

IBD inflammatory bowel disease, SLE systemic lupus erythematosus, MS multiple sclerosis, RA rheumatoid arthritis, GA gout arthritis, T1D type 1 diabetes, AIH autoimmune hepatitis, BMDM bone marrow derived macrophage, MDSC myeloid-derived suppressor cell, EAE experimental autoimmune encephalomyelitis, DSS dextran sulfate sodium, LPS lipopolysaccharide, DAI disease activities index, ↑ up, ↓ down.