Protein NLRP3

URN urn:agi-llid:9558
Total Entities 0
Connectivity 4753
Name NLRP3
Description NLR family, pyrin domain containing 3
Notes This gene encodes a pyrin-like protein containing a pyrin domain, a nucleotide-binding site (NBS) domain, and a leucine-rich repeat (LRR) motif. This protein interacts with the apoptosis-associated speck-like protein PYCARD/ASC, which contains a caspase recruitment domain, and is a member of the NALP3 inflammasome complex. This complex functions as an upstream activator of NF-kappaB signaling, and it plays a role in the regulation of inflammation, the immune response, and apoptosis. Mutations in this gene are associated with familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), chronic infantile neurological cutaneous and articular (CINCA) syndrome, and neonatal-onset multisystem inflammatory disease (NOMID). Multiple alternatively spliced transcript variants encoding distinct isoforms have been identified for this gene. Alternative 5' UTR structures are suggested by available data; however, insufficient evidence is available to determine if all of the represented 5' UTR splice patterns are biologically valid. [provided by RefSeq, Oct 2008]
ObjectType Protein

Pathway NOD-like Receptors
Dectin-1 (CLEC7A) Signaling
Toll-like Receptor Independent Sterile Inflammation
Proteins Involved in Myocarditis
Inflammatory Reaction in Acne Vulgaris
Apoptotic Keratinocytes Clearance Recession in Systemic Lupus Erythematosus
Dendritic Cell Activation in Systemic Lupus Erythematosis
Plasmacytoid Dendritic Cell in Diabetes Mellitus Type 1
Macrophage Function in Diabetes Mellitus Type 1
Conventional Dendritic Cell Role in Diabetes Mellitus Type 1
Proteins Involved in non-Alcoholic Fatty Liver Disease
Crohn's Disease
Macrophage Dysfunction
Dendritic Cell Function in Ulcerative Colitis
Epithelial Cell in the Innate Immune Response in Ulcerative Colitis
Antigen-Presenting Cell Role in Asthma
Proteins Involved in Gout
Monocytes Function in Gout
Dendritic Cell Dysfunction in Psoriatic Arthritis
Proteins Involved in Periodontitis
PAPA Syndrome
Blau Syndrome
Cryopyrin (NLPR3) Associated Periodic Syndromes
Familial Cold Autoinflammatory Syndrome 2
Familial Mediterranean Fever
Majeed Syndrome
Proteins Involved in Age-Related Macular Degeneration
Immune System Role in Otitis Media
Alveolar Macrophages Dysfunction in COPD
Elevated Receptors -> Expression Targets in Mouth
Asbestosis- and Silicosis-Associated Inflammation Provokes Cancer Development
Proteins/Ions Transporters -> Expression Targets in Lymphoid System and Blood
P2RX7 -> Expression Targets in Nerve Tissue
e
pathway 4
pprarg neg, uknown targets, ps-positive
Model of PPARG signaling in psoriasis
Model of PPARG related pathways in psoriasis (short version)
sars-cov binding
sars-cov no binding
Dendritic Cell Activation in COVID19
Overview. Diseases of the digestive system (chapter 10)
1_Inflammation in keratinocytes in the pathology of acne
1_Insufficient activation of immune response in the middle ear epithelium cells in otitis media
3_Role of alveolar macrophages in pulmonary tissue destruction in COPD
1_Th2 cell response in asthma
2_Paneth cell dysfunction in Crohn's disease
1_Defects in response to pathogens in the gut promotes inflammation in Crohn's disease
2_Intestine epithelial cells drive immunological responses in ulcerative colitis
4_1_Polymorphisms associated with inflammatory bowel diseases
1_Antigen-presenting cells promote islet dysfunction and immune system activation in T1D
Figure 4_Diabetes mellitus Type 1: conventional dendritic cell
drug-target pairs direct regulation

Group Genes with Mutations Associated with Vitiligo
binding partnetrs for SARSs
other partners for SARS

MedScan ID 9558

LocusLink ID 9558
114548
216799
287362

Alias deafness, autosomal dominant 34
OTTHUMP00000038410
AII/AVPs
NACHT, LRR and PYD containing protein 3
CLR1.1
Familial cold autoinflammatory syndrome gene
FCAS1
PYRIN-containing APAF1-like protein I
C1orf7
Muckle-Wells syndrome protein
neuronal apoptosis inhibitor protein 3
Muckle-Wells syndrome gene
chromosome 1 open reading frame VII
NACHT domain-, leucine-rich repeat-, and PYD-containing protein 3
RP23-210M6.4
cold-induced autoinflammatory syndrome 1 protein
familial cold urticaria gene
KEFH
FLJ95925
NALP3
nucleotide-binding oligomerization domain, leucine rich repeat and pyrin domain containing 3
Mmig1
NACHT, LRR and PYD containing protein III
NLR family, pyrin domain containing 3
AGTAVPRL
MWS
OTTMUSP00000005970
cryopyrin, NACHT, LRR and PYD domains-containing protein 3
PYPAF I
cryopyrin
OTTHUMP00000038408
NACHT, LRR and PYD domains-containing protein 3
mast cell maturation-associated-inducible protein 1
mast cell maturation inducible protein 1
NACHT domain leucine rich repeat and pyrin domain containing protein 3
chromosome 1 open reading frame 7
cold autoinflammatory syndrome 1 homolog
AII/AVP
cold induced autoinflammatory syndrome 1 protein
OTTHUMP00000038411
cold-induced autoinflammatory syndrome-1 protein
DFNA34
cold autoinflammatory syndrome 1
OTTHUMP00000038409
PYRIN-containing APAF1-like protein 1
angiotensin/vasopressin receptor AII/AVP-like
caterpiller protein 1.1
FCAS-1
MGC129375
PYPAF1
aii/avp receptor-like
NLRP3
cold autoinflammatory syndrome I
CIAS1
neuronal apoptosis inhibitor protein III
NACHT/LRR/pyrin domain-containing protein 3
Mmig I
CIAS I
AII
AVP
FCU
FCAS
cold autoinflammatory syndrome 1 protein
cryopyrin, NACHT, LRR and PYD domains - containing protein 3
cold autoinflammatory syndrome 1 protein homolog

GO ID 0005524
0042834
0043565
0008134
0044546
0006919
0006915
0071222
0071224
0002374
0006952
0050830
0051607
0009595
0006954
0045087
0032611
0050701
0032621
0032088
1901223
0002674
0050728
0050713
0051092
2000321
2000553
0045630
0043280
0050718
0032736
0032753
0032754
0045944
0002830
0051259
0016579
0007165
0000139
0072559
0005737
0005829
0005783
0005576
0005634
0000166
0002376
0050727
0061702
0005623
0006917
0035872
0006351
0006355
0005515
0042347
0009617

KEGG ID rno:287362
hsa:114548
mmu:216799

Organism Homo sapiens {Organism urn:agi-taxid:9606}
Mus musculus {Organism urn:agi-taxid:10090}
Rattus norvegicus {Organism urn:agi-taxid:10116}
Homo sapiens
Mus musculus
Rattus norvegicus

Mouse chromosome position 11

OMIM ID 606416
607115
617772
191900
120100
148200

Rat chromosome position 10q22

Hugo ID 16400
HGNC:16400

Human chromosome position 1q44

Swiss-Prot Accession Q96P20
Q96P20.3
Q8R4B8
Q8R4B8.1
D4A523
D4A523.2
A0A024R5Q0
B2RC97
B7ZKS9
B7ZKT2
B7ZKT3
O75434
Q17RS2
Q59H68
Q5JQS8
Q5JQS9
Q6TG35
Q8TCW0
Q8TEU9
Q8WXH9
Q1JQ87
Q1JQ88
Q6JEL0
T1W2H6
Q65Z67
Q5SXI6

GenBank ID NC_000001
NM_001127461
NP_001120933
NM_001079821
NP_001073289
XM_017000183
XP_016855672
XM_017000184
XP_016855673
XM_017000182
XP_016855671
XM_017000181
XP_016855670
XM_011544048
XP_011542350
XM_024452862
XP_024308630
XM_024452874
XP_024308642
NM_004895
NP_004886
NM_001243133
NP_001230062
NM_001127462
NP_001120934
NM_183395
NP_899632
NG_007509
AB120959
BAD51451
AB120960
BAD51452
AB120961
BAD51453
AB120962
BAD51454
AC104335
AH011140
AAL12497
AAL12498
AL606804
CH471148
EAW77183
EAW77184
EAW77185
EAW77186
EAW77187
MG557564
AXK59206
AB208891
BAD92128
AF054176
AAC39910
AF410477
AAL33908
AF418985
AAL14640
AF420469
AAL65136
AF427617
AAL33911
AF468522
AAL78632
AK027194
AK314998
BAG37494
AL601856
AY092033
AAM14669
AY422168
AAQ98889
BC117211
AAI17212
BC143359
AAI43360
BC143362
AAI43363
BC143363
AAI43364
DA244290
Q96P20
NC_000077
XM_006532857
XP_006532920
XR_388400
XM_006532858
XP_006532921
NM_145827
NP_665826
NM_001359638
NR_153314
AH013321
AAR03541
AH013322
AAR03542
AH013323
AAR03543
AL592522
AF486632
AAL90874
AW121591
AY337285
AAR03540
AY355340
AAR14737
AY495376
AAS75794
AY495377
AAS75795
BC116174
AAI16175
BC116175
AAI16176
BY186774
BY722759
KF032621
AGU01502
Q8R4B8
NC_005109
XM_017597078
XP_017452567
XM_006246453
XP_006246515
XM_006246458
XP_006246520
XM_006246455
XP_006246517
XM_006246457
XP_006246519
NM_001191642
NP_001178571
AC_000078
AABR07029714
AABR07029715
AABR07072123
AAHX01064203
AAHX01064204
AC123316
FQ231619
D4A523
CAI17154
CAI17153
CAI17155
XP_006246518
XP_006246516
AY337302
AY337303
AY337300
AY337301
AY337306
AY337304
AY337305
NW_001071914
AAHY01147154
CAI24551
CAI24552
XM_006711733
XM_005273036
XM_005273037
XM_005273038
NC_018912
XP_006711796
AY051113
AY051112
XM_006246454
XM_006246456
XP_005273093
XP_005273094
XP_005273095
AY051115
AY051114
AY051117
AY051116
XM_011544049
XP_006532922
XM_011544051
XM_011544050
XM_011544053
XM_011544052
XM_011544055
XM_011544054
AMYH02003133
AMYH02003132
AY056060
AY337289
AY337287
AY337288
AY337286
AABR06064074
AABR06064075
XP_011542356
XP_011542357
XP_011542354
XP_011542355
XP_011542352
XP_011542353
XP_011542351
AY056059
AC_000133
XM_006532859
AY337294
AY337295
AY337292
AY337293
AY337290
AY337291
ABBA01031787
ABBA01031788
AY337298
AY337299
AY337296
AY337297
NT_167186
NW_001838554
NW_927572
AC_000044
CR609473
Q65Z67
NT_096135
Q5SXI6
XM_220513
XP_220513
NW_047334
NW_001084656
XM_001075398
XP_001075398

Swiss-Prot ID NLRP3_RAT
NLRP3_HUMAN
NLRP3_MOUSE
NALP3_HUMAN
NALP3_MOUSE
B2RC97_HUMAN
B7ZKT2_HUMAN
B7ZKT3_HUMAN

Cell Localization Cytoplasm
Cytosol
Inflammasome
Endoplasmic reticulum
Secreted
Nucleus
Golgi apparatus membrane
Cytoplasm, cytosol

Ensembl ID ENSG00000162711
ENSP00000375704.3
ENST00000391828.7
ENSP00000355453.2
ENST00000366497.6
ENSP00000375703.2
ENST00000391827.2
ENSP00000337383.3
ENST00000336119.7
ENSP00000294752.3
ENST00000348069.6
ENSMUSG00000032691
ENSMUSP00000098707.2
ENSMUST00000101148.8
ENSMUSP00000078440.3
ENSMUST00000079476.9
ENSRNOG00000003170
ENSRNOP00000004280.3
ENSRNOT00000004280.5
ENSP00000375704
ENSMUST00000079476
ENSP00000375703
ENST00000366497
ENSMUSP00000098707
ENSP00000355453
ENST00000348069
ENST00000336119
ENST00000391828
ENST00000391827
ENSP00000337383
ENSMUSP00000078440
ENSRNOT00000004280
ENSRNOP00000004280
ENSP00000294752
ENSMUST00000101148

MGI ID MGI:2653833
2653833

RGD ID 1308314

Unigene ID Mm.54174
Hs.159483
Rn.214177

Homologene ID 3600

IPI ID IPI00374129
IPI00153873
IPI00556137
IPI00742837
IPI00620897
IPI00553033
IPI00451585
IPI00621784
IPI00620168
IPI00198971
IPI00777914
IPI00777199