KEGG   PATHWAY: hsa04621Help
Entry
hsa04621                    Pathway                                

Name
NOD-like receptor signaling pathway - Homo sapiens (human)
Description
Specific families of pattern recognition receptors are responsible for detecting various pathogens and generating innate immune responses. The intracellular NOD-like receptor (NLR) family contains more than 20 members in mammals and plays a pivotal role in the recognition of intracellular ligands. NOD1 and NOD2, two prototypic NLRs, sense the cytosolic presence of the bacterial peptidoglycan fragments that escaped from endosomal compartments, driving the activation of NF-{kappa}B and MAPK, cytokine production and apoptosis. On the other hand, a different set of NLRs induces caspase-1 activation through the assembly of multiprotein complexes called inflammasomes. These NLRs include NALP1, NALP3 and Ipaf. The inflammasomes are critical for generating mature proinflammatory cytokines in concert with Toll-like receptor signaling pathway.
Class
Organismal Systems; Immune system
BRITE hierarchy
Pathway map
NOD-like receptor signaling pathway
hsa04621

All organismsOrtholog table
Disease
H00282  
Cryopyrin associated periodic syndrome (CAPS)
H00285  
Blau syndrome
H00286  
Crohn's disease
H00287  
Pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome
H00288  
Familial Mediterranean fever (FMF)
H00882  
Cocoon syndrome
H01109  
Chronic mucocutaneous candidiasis (CMC)
H01309  
Sarcoidosis, early-onset
H01372  
Vitiligo
Drug
D06635  
Rilonacept (USAN/INN)
D08978  
Pralnacasan (USAN/INN)
D09315  
Canakinumab (genetical recombination) (JAN)
D09375  
Retaspimycin (USAN/INN)
D09376  
Retaspimycin hydrochloride (USAN)
D09911  
Gevokizumab (USAN/INN)
D10416  
Belnacasan (USAN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
10392  
NOD1; nucleotide binding oligomerization domain containing 1 [KO:K08727]
64127  
NOD2; nucleotide binding oligomerization domain containing 2 [KO:K10165]
7205  
TRIP6; thyroid hormone receptor interactor 6 [KO:K12792]
8767  
RIPK2; receptor interacting serine/threonine kinase 2 [KO:K08846] [EC:2.7.11.1]
7189  
TRAF6; TNF receptor associated factor 6 [KO:K03175] [EC:2.3.2.27]
8517  
IKBKG; inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma [KO:K07210]
1147  
CHUK; conserved helix-loop-helix ubiquitous kinase [KO:K04467] [EC:2.7.11.10]
3551  
IKBKB; inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase beta [KO:K07209] [EC:2.7.11.10]
4793  
NFKBIB; NFKB inhibitor beta [KO:K02581]
4792  
NFKBIA; NFKB inhibitor alpha [KO:K04734]
4790  
NFKB1; nuclear factor kappa B subunit 1 [KO:K02580]
5970  
RELA; RELA proto-oncogene, NF-kB subunit [KO:K04735]
3576  
CXCL8; C-X-C motif chemokine ligand 8 [KO:K10030]
6347  
CCL2; C-C motif chemokine ligand 2 [KO:K14624]
6352  
CCL5; C-C motif chemokine ligand 5 [KO:K12499]
7124  
TNF; tumor necrosis factor [KO:K03156]
3569  
IL6; interleukin 6 [KO:K05405]
6885  
MAP3K7; mitogen-activated protein kinase kinase kinase 7 [KO:K04427] [EC:2.7.11.25]
10454  
TAB1; TGF-beta activated kinase 1/MAP3K7 binding protein 1 [KO:K04403]
23118  
TAB2; TGF-beta activated kinase 1/MAP3K7 binding protein 2 [KO:K04404]
257397  
TAB3; TGF-beta activated kinase 1/MAP3K7 binding protein 3 [KO:K12793]
5594  
MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
5595  
MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
5599  
MAPK8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
5602  
MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
5601  
MAPK9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
5600  
MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
6300  
MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
5603  
MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
1432  
MAPK14; mitogen-activated protein kinase 14 [KO:K04441] [EC:2.7.11.24]
64170  
CARD9; caspase recruitment domain family member 9 [KO:K12794]
841  
CASP8; caspase 8 [KO:K04398] [EC:3.4.22.61]
10910  
SUGT1; SGT1 homolog, MIS12 kinetochore complex assembly cochaperone [KO:K12795]
55914  
ERBIN; erbb2 interacting protein [KO:K12796]
84674  
CARD6; caspase recruitment domain family member 6 [KO:K12797]
329  
BIRC2; baculoviral IAP repeat containing 2 [KO:K16060]
330  
BIRC3; baculoviral IAP repeat containing 3 [KO:K16060]
7128  
TNFAIP3; TNF alpha induced protein 3 [KO:K11859] [EC:3.4.19.12]
22861  
NLRP1; NLR family, pyrin domain containing 1 [KO:K12798]
29108  
PYCARD; PYD and CARD domain containing [KO:K12799]
834  
CASP1; caspase 1 [KO:K01370] [EC:3.4.22.36]
838  
CASP5; caspase 5 [KO:K04395] [EC:3.4.22.58]
114548  
NLRP3; NLR family, pyrin domain containing 3 [KO:K12800]
22900  
CARD8; caspase recruitment domain family member 8 [KO:K12801]
260434  
PYDC1; pyrin domain containing 1 [KO:K12802]
4210  
MEFV; Mediterranean fever [KO:K12803]
9051  
PSTPIP1; proline-serine-threonine phosphatase interacting protein 1 [KO:K12804]
3320  
HSP90AA1; heat shock protein 90kDa alpha family class A member 1 [KO:K04079]
3326  
HSP90AB1; heat shock protein 90kDa alpha family class B member 1 [KO:K04079]
7184  
HSP90B1; heat shock protein 90kDa beta family member 1 [KO:K09487]
58484  
NLRC4; NLR family, CARD domain containing 4 [KO:K12805]
59082  
CARD18; caspase recruitment domain family member 18 [KO:K12806]
4671  
NAIP; NLR family, apoptosis inhibitory protein [KO:K12807]
3553  
IL1B; interleukin 1 beta [KO:K04519]
3606  
IL18; interleukin 18 [KO:K05482]
2919  
CXCL1; C-X-C motif chemokine ligand 1 [KO:K05505]
2920  
CXCL2; C-X-C motif chemokine ligand 2 [KO:K05505]
Compound
C16060  
Defensin beta-2
Reference
  Authors
Shaw MH, Reimer T, Kim YG, Nunez G
  Title
NOD-like receptors (NLRs): bona fide intracellular microbial sensors.
  Journal
Curr Opin Immunol 20:377-82 (2008)
Reference
  Authors
Kanneganti TD, Lamkanfi M, Nunez G
  Title
Intracellular NOD-like receptors in host defense and disease.
  Journal
Immunity 27:549-59 (2007)
Reference
  Authors
Franchi L, Warner N, Viani K, Nunez G
  Title
Function of Nod-like receptors in microbial recognition and host defense.
  Journal
Immunol Rev 227:106-28 (2009)
Reference
  Authors
Tattoli I, Travassos LH, Carneiro LA, Magalhaes JG, Girardin SE
  Title
The Nodosome: Nod1 and Nod2 control bacterial infections and inflammation.
  Journal
Semin Immunopathol 29:289-301 (2007)
Reference
  Authors
da Silva Correia J, Miranda Y, Leonard N, Hsu J, Ulevitch RJ
  Title
Regulation of Nod1-mediated signaling pathways.
  Journal
Cell Death Differ 14:830-9 (2007)
Reference
  Authors
Reardon C, Mak TW
  Title
cIAP proteins: keystones in NOD receptor signal transduction.
  Journal
Immunity 30:755-6 (2009)
Reference
  Authors
Fritz JH, Ferrero RL, Philpott DJ, Girardin SE
  Title
Nod-like proteins in immunity, inflammation and disease.
  Journal
Nat Immunol 7:1250-7 (2006)
Reference
  Authors
Rietdijk ST, Burwell T, Bertin J, Coyle AJ
  Title
Sensing intracellular pathogens-NOD-like receptors.
  Journal
Curr Opin Pharmacol 8:261-6 (2008)
Reference
  Authors
Hsu YM, Zhang Y, You Y, Wang D, Li H, Duramad O, Qin XF, Dong C, Lin X
  Title
The adaptor protein CARD9 is required for innate immune responses to intracellular pathogens.
  Journal
Nat Immunol 8:198-205 (2007)
Reference
  Authors
Kufer TA, Kremmer E, Banks DJ, Philpott DJ
  Title
Role for erbin in bacterial activation of Nod2.
  Journal
Infect Immun 74:3115-24 (2006)
Reference
  Authors
Franchi L, Eigenbrod T, Munoz-Planillo R, Nunez G
  Title
The inflammasome: a caspase-1-activation platform that regulates immune responses and disease pathogenesis.
  Journal
Nat Immunol 10:241-7 (2009)
Reference
  Authors
Martinon F, Gaide O, Petrilli V, Mayor A, Tschopp J
  Title
NALP inflammasomes: a central role in innate immunity.
  Journal
Semin Immunopathol 29:213-29 (2007)
Reference
  Authors
Martinon F, Tschopp J
  Title
Inflammatory caspases: linking an intracellular innate immune system to autoinflammatory diseases.
  Journal
Cell 117:561-74 (2004)
Reference
  Authors
Martinon F, Mayor A, Tschopp J
  Title
The inflammasomes: guardians of the body.
  Journal
Annu Rev Immunol 27:229-65 (2009)
Reference
  Authors
Lamkanfi M, Dixit VM
  Title
Inflammasomes: guardians of cytosolic sanctity.
  Journal
Immunol Rev 227:95-105 (2009)
Reference
  Authors
Benko S, Philpott DJ, Girardin SE
  Title
The microbial and danger signals that activate Nod-like receptors.
  Journal
Cytokine 43:368-73 (2008)
KO pathway
 

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