ライフサイエンスコーパス: cryopyrin

1 ine toxin, was also found to be dependent on cryopyrin.

2 nucleotide-binding oligomerization domain of cryopyrin.

3 uggesting that inhibition occurs upstream of Cryopyrin.

4 8 +/- 0.52 microm but does not interact with Cryopyrin.

5 phages but unimpaired in macrophages lacking Cryopyrin.

6 Here, we investigated whether NLRP3/cryopyrin, a component of the inflammasome, participates

7 This protein thus resembles Cryopyrin, a protein implicated in hereditary hyperinfla

8 flammatory syndrome 1 (CIAS1) gene, encoding cryopyrin, a protein that regulates inflammation.

9 t here that similar to several NLRP3 (NALP3, cryopyrin)-activating stimuli, LT activation of the NLRP

10 is the first identified compound to prevent Cryopyrin activation and microbial ligand-, DAMP-, and c

11 The induction of cryopyrin activity by enforced oligomerization in THP-1

12 Inappropriate Cryopyrin activity has been incriminated in the pathogen

13 Cryopyrin (also called Nalp3), the product of CIAS1, is

14 ASC also interacts with the adaptor cryopyrin (also known as NALP3 or CIAS1).

15 by mutations in the CIAS1 gene that encodes cryopyrin, an adaptor protein involved in activation of

16 NLRP3 encodes cryopyrin, an intracellular "molecular sensor" that form

17 Cryopyrin and ASC are essential for caspase-1 activation

18 ese results identify a mechanism mediated by cryopyrin and ASC that links dsRNA and viral infection t

19 Similarly, Cryopyrin and ASC were required for activation of caspas

20 Thus, cryopyrin and caspase-1 are central to both innate immun

21 ers the activation of a NALP3 (also known as cryopyrin and NLRP3)-independent inflammasome, which act

22 The pyrin and NACHT domains of Cryopyrin and other NALP proteins are highly conserved a

23 ASC and PAN1, thereby blocking formation of cryopyrin and PAN1-containing inflammasomes, activation

24 ntaining a caspase-recruitment domain (ASC), cryopyrin, and caspase-1, localized to the granulation t

25 eral NOD-LRR proteins, including human Nod2, Cryopyrin, and CIITA, as well as mouse Naip5, is associa

26 Nod1, Nod2, Cryopyrin, and Ipaf have been implicated in protective i

27 and H3 helices, may be the binding site for Cryopyrin, and the interaction with Cryopyrin may induce

28 and PYD domains-containing protein 3 (NALP3; cryopyrin), apoptosis-associated speck-like CARD-domain

29 Activating mutations in cryopyrin are associated with familial cold autoinflamma

30 Human and mouse Cryopyrin are highly conserved and consist of three func

31 Mutations in cryopyrin are hypothesized to result in abnormal secreti

32 Mutations in CIAS1/cryopyrin are linked to several autoinflammatory disease

33 ions in another PYRIN domain protein, termed cryopyrin, are responsible for three clinically defined

34 d mutants, identifying nucleotide binding by cryopyrin as a potential target for antiinflammatory pha

35 arly-onset autoinflammatory syndromes, CAPS (cryopyrin associated periodic syndromes) and DIRA (defic

36 s consistent with symptoms observed in human Cryopyrin-associated diseases.

37 12 with adult-onset Still's disease, 7 with cryopyrin-associated periodic disease, 9 with Waldenstro

38 al acquired inflammatory diseases as well as cryopyrin-associated periodic fever syndromes (CAPS) cau

39 IL-1beta secretion from LPS-primed PBMCs of cryopyrin-associated periodic fever syndromes patients w

40 is pathogenic in inherited disorders such as cryopyrin-associated periodic syndrome (CAPS) and comple

41 The cryopyrin-associated periodic syndrome (CAPS) is a rare

42 Cryopyrin-associated periodic syndrome (CAPS) patients w

43 pare the biomarkers in sJIA to biomarkers in cryopyrin-associated periodic syndrome (CAPS).

44 The latter include cryopyrin-associated periodic syndrome and Schnitzler's

45 nockin mice, which carry a mutation found in cryopyrin-associated periodic syndrome patients, was sup

46 inflammasome-related genes in patients with cryopyrin-associated periodic syndromes (CAPS) and famil

47 ain-of-function mutations in NLRP3 cause the cryopyrin-associated periodic syndromes (CAPS) and trigg

48 Cryopyrin-associated periodic syndromes (CAPS) are cause

49 cytokines IL-1beta and IL-18, leading to the cryopyrin-associated periodic syndromes (CAPS) disease s

50 Additionally, QUC inhibited IL-1beta in Cryopyrin-Associated Periodic Syndromes (CAPS) macrophag

51 tes from patients affected by NLRP3-mediated cryopyrin-associated periodic syndromes (CAPS) release g

52 p.D303N mutant form of NLRP3 associated with cryopyrin-associated periodic syndromes (CAPS) stimulate

53 In contrast, patients with cryopyrin-associated periodic syndromes (CAPS), a rare m

54 lammatory diseases collectively known as the cryopyrin-associated periodic syndromes (CAPS).

55 s, arthralgia, and bone and muscle pain with cryopyrin-associated periodic syndromes (CAPS).

56 ectrum of autoinflammatory diseases known as cryopyrin-associated periodic syndromes (CAPS).

57 P3/CIAS1 mosaicism is a significant cause of cryopyrin-associated periodic syndromes (CAPS).

58 ic autoinflammatory disease spectrum, termed cryopyrin-associated periodic syndromes (CAPS).

59 cause the autoinflammatory disease spectrum cryopyrin-associated periodic syndromes (CAPS).

60 LRP3 cause the disease spectrum known as the cryopyrin-associated periodic syndromes (CAPS).

61 ndrome, and familial Mediterranean fever and cryopyrin-associated periodic syndromes allow insights i

62 illnesses, given rise to the concept of the cryopyrin-associated periodic syndromes as a disease spe

63 Cryopyrin-associated periodic syndromes respond to treat

64 hese diseases which have been grouped as the cryopyrin-associated periodic syndromes result from defe

65 associated periodic syndrome, 6 patients had cryopyrin-associated periodic syndromes, and 4 patients

66 ed that IL-1beta oversecretion is pivotal in cryopyrin-associated periodic syndromes, and that IL-1 i

67 ulinemia D with periodic fever syndrome, and cryopyrin-associated periodic syndromes.

68 ally efficacious in rheumatoid arthritis and cryopyrin-associated periodic syndromes.

69 owing to mutations in the NLRP3 gene, causes cryopyrin-associated periodic syndromes.

70 We demonstrate that purified cryopyrin binds ATP, dATP, and ATP-agarose, but not CTP,

71 duced by MDP and ATP required pannexin-1 and Cryopyrin but was independent of Nod2.

72 phages, cells deficient in the gene encoding cryopyrin (Cias1-/-) activated caspase-1 and secreted no

73 ponse system called the Nalp3 (also known as cryopyrin, CIAS1 or NLRP3) inflammasome.

74 ning 3" (NLRP3) inflammasome, also known as "cryopyrin," "cold-induced autoinflammatory syndrome 1" (

75 ermore, we show that Toll-like receptors and cryopyrin control the secretion of IL-1beta and IL-18 th

76 Here we show the effect of cryopyrin deficiency on inflammasome function and immune

77 d protein kinases (MAPKs) were unaffected by cryopyrin deficiency.

78 Despite the effect on innate immunity, cryopyrin-deficiency was not associated with any obvious

79 Here we show that cryopyrin-deficient macrophages cannot activate caspase-

80 on of hyaluronan to macrophages derived from cryopyrin-deficient mice increased release of Cxcl2 but

81 ced the secretion of IL-1beta and IL-18 in a cryopyrin-dependent manner.

82 Further, these results indicate that cryopyrin disease-associated mutants are constitutively

83 generated constructs to express three common cryopyrin disease-associated mutations, R260W, D303N, an

84 er candidate genes were sequenced, models of cryopyrin domains were constructed using structurally ho

85 chanism of hyaluronan-mediated activation of cryopyrin, elements of the hyaluronan recognition proces

86 Cryopyrin expression is also very similar in human and m

87 omponents zymosan and mannan require ASC and Cryopyrin for caspase-1 activation and IL-1beta secretio

88 Cryopyrin forms a multi-protein complex termed 'the infl

89 Mutations in the NALP3/CIAS1/cryopyrin gene are linked to three autoinflammatory diso

90 luronan catabolism trigger the intracellular cryopyrin --> IL-1beta pathway.

91 Involvement of cryopyrin in activation of caspase 1 and NF-kappaB signa

92 Concurrent with the role of Cryopyrin in endotoxemia, glyburide significantly delays

93 These results reveal a critical role for cryopyrin in host defence through bacterial RNA-mediated

94 en proposed, including the role of pyrin and cryopyrin in regulating inflammation.

95 flagellin, induced caspase-1 activation via cryopyrin in the absence of pannexin-1 activity or ATP s

96 this issue of Immunity, a critical role for cryopyrin in the caspase-1/IL-1beta axis and reveal a br

97 ides evidence for distinct roles of Nod2 and Cryopyrin in the regulation of MDP-induced caspase-1 act

98 tural effect of disease-causing mutations on cryopyrin, in order to gain better understanding of the

99 rtain PAAD-family proteins such as Pyrin and Cryopyrin increases NF-kappaB activity, ASC has an inhib

100 retion and suggest an important role for the Cryopyrin inflammasome during fungal infections.

101 These findings reveal a function of the cryopyrin inflammasome in healing responses.

102 (MSU) crystals intracellularly activate the cryopyrin inflammasome is unknown.

103 ding cassette transporters also activate the Cryopyrin inflammasome normally.

104 RNA, are thought to trigger assembly of the cryopyrin inflammasome resulting in caspase-1 activation

105 h Sendai and influenza viruses activates the cryopyrin inflammasome.

106 t also other known stimuli that activate the cryopyrin inflammasome.

107 n induce activation of caspase-7 through the Cryopyrin inflammasome.

108 s drug glyburide prevented activation of the Cryopyrin inflammasome.

109 istic link between bacterial stimuli and the cryopyrin inflammasome.

110 taining-3 (Nlrp3, but also known as Nalp3 or cryopyrin) inflammasome are implicated in recognizing ce

111 interactions, the description of the NALP3 (cryopyrin) inflammasome as a macromolecular complex for

112 The NLRP3 (NALP3, cryopyrin) inflammasome, a key component of the innate i

113 ing the caspase-1-activating NLRP3 (NALP3 or cryopyrin) inflammasome, which results in cleavage and s

114 P2X7 and cryopyrin inhibition (using silencing RNA or a pharmacol

115 Cryopyrin is essential for caspase-1 activation triggere

116 Therefore, cryopyrin is essential for inflammasome activation in re

117 ther the leucine-rich repeat (LRR) domain of cryopyrin is required for MSU crystal-induced inflammati

118 Cryopyrin-knockout lacZ (Cryo(-Z/-Z)) mice and mice with

119 ut lacZ (Cryo(-Z/-Z)) mice and mice with the cryopyrin LRR domain deleted and fused to the lacZ repor

120 These data suggest CIAS1/cryopyrin may act as a key regulator of inflammation, in

121 site for Cryopyrin, and the interaction with Cryopyrin may induce the dissociation of POP1 from ASC_P

122 Here we demonstrate that cryopyrin-mediated caspase-1 activation proceeds indepen

123 Thus, cryopyrin-mediated IL-1beta secretion requires ASC in mo

124 n inner surface of the hexameric ring in the cryopyrin model, consistent with the hypothesis that the

125 All cryopyrin mutant proteins tested were found to induce po

126 However, cryopyrin mutations are found only in 50% of patients wi

127 into potential molecular mechanisms by which cryopyrin mutations can inappropriately activate an infl

128 The mechanism by which cryopyrin mutations cause inflammatory disease remains e

129 ociated autoinflammatory syndrome-associated Cryopyrin mutations, thus suggesting that inhibition occ

130 We demonstrate that NLRP3 (cryopyrin, NALP3) is the primary NLR required for IL-1be

131 roles for the NLR (NBD-LRR) protein, NLRP3 (cryopyrin, NALP3), and its adaptor apoptotic speck prote

132 crophages lacking the NOD-like (NLR) protein Cryopyrin/Nalp3 and in wild type macrophages pretreated

133 The CATERPILLER protein cryopyrin/NALP3 regulates IL-1beta processing by assembl

134 RNA) and viral infection that is mediated by Cryopyrin/Nalp3.

135 Cryopyrin/NALP3/NLRP3 is an essential component of infla

136 Missense mutations in cryopyrin (NLRP3) result in a hyperactive inflammasome t

137 ns in proteins that are either homologous to cryopyrin or involved in the caspase 1/interleukin-1beta

138 n family, pyrin domain containing 3 (NLRP3) (cryopyrin or NALP3) as a prominent player.

139 ral RNA was abrogated in macrophages lacking cryopyrin or the adaptor ASC (apoptosis-associated speck

140 -1 activation, stimulation of the pannexin-1-cryopyrin pathway by several intracellular bacteria was

141 Hence, the LRR domain of cryopyrin plays a role in mediating MSU crystal-induced

142 e caused by mutations in CIAS1, encoding the cryopyrin protein.

143 n of the PAAD/PYRIN family proteins pyrin or cryopyrin/PYPAF1/NALP3 individually inhibits IL-1beta se

144 events linking bacterial products and ATP to cryopyrin remain unclear.

145 Mice with a targeted deletion in cryopyrin showed a normal increase in Cxcl2 in response

146 ptor protein previously suggested to mediate cryopyrin signaling.

147 at hyaluronan works through IL-1beta and the cryopyrin system to signal sterile inflammation.

148 /IL-1beta axis and reveal a broader role for cryopyrin than anticipated is uncovered.

149 Cryopyrin, the protein that is altered in FCAS, is one o

150 that the mutations disrupt a closed form of cryopyrin, thus potentiating inflammasome assembly.

151 onocytogenes, however, required both ASC and cryopyrin to activate caspase-1 and secrete IL-1beta.

152 Finally, cryopyrin was required for IL-1beta production in respon

153 In the absence of stimuli, wild-type cryopyrin was unable to bind to ASC, whereas the three m

154 CIAS1 codes for the protein Cryopyrin, which appears to play a role in innate immune

155 te crystals stimulate IL-1beta secretion via cryopyrin, which led to the addition of gout to the spec

156 These results further link mutations in cryopyrin with abnormal caspase-1 activation, and suppor