ライフサイエンスコーパス: FMF

1 FMF attacks are unique in their sensitivity to the micro

2 FMF is associated with increased risk of mortality after

3 FMF was associated with more than twofold increased risk

4 A FMF-knock-in mouse strain that expresses chimeric pyrin

5 ifferentiate between indolent and aggressive FMF and confirm the existence of a subgroup of FMF with

6 Distinction between indolent and aggressive FMF may have important therapeutic consequences but is h

7 imated 70% increase in the odds of having an FMF attack on the second day (95% confidence interval: 1

8 02; 5-year and 10-year OS, 55% and 28%); and FMF presenting with extracutaneous disease (group C; n =

9 ing mediators, we studied PBMC from HIDS and FMF patients in the inactive phase of disease.

10 roduction by unstimulated PBMC from HIDS and FMF patients supports the likelihood of different pathog

11 ctivating agents and show that wild-type and FMF Pyrin are differentially controlled by microtubules.

12 was not overrepresented in Armenian or Arab FMF carriers.

13 g factors in attacks in a sample of Armenian FMF patients in Yerevan, Armenia, where 104 patients con

14 nic causes of inflammatory diseases, such as FMF, PAPA, TRAPS, and HIDS, has elucidated the pathophys

15 t improvement of clinical parameters in both FMF-CP and CP groups after periodontal treatment.

16 evealed a biochemical pathway common to both FMF and PAPA.

17 /IL-1beta construct, an effect diminished by FMF-associated B30.2 mutations and in B30.2 deletion mut

18 the interaction, and binding was reduced by FMF-associated B30.2 mutations.

19 We recently identified the gene causing FMF, designated MEFV, and found it to be expressed in ma

20 substantial number of patients with clinical FMF possess only 1 demonstrable MEFV mutation.

21 om 13 patients with FMF with generalized CP (FMF-CP), 15 systemically healthy patients with generaliz

22 REDD1) is significantly overexpressed during FMF attacks.

23 Here we explore the capability of employing FMF for long distance quantum communication.

24 tory diseases: familial Mediterranean fever (FMF) and hyperimmunoglobulinemia D syndrome (HIDS).

25 Familial Mediterranean fever (FMF) and the hyperimmunoglobulinemia D and periodic feve

26 ho experienced familial Mediterranean fever (FMF) and their response to non-surgical periodontal ther

27 ome (HIDS) and familial Mediterranean fever (FMF) are both characterized by attacks of periodic fever

28 of the common familial Mediterranean fever (FMF) gene MEFV mutations were excluded.

29 Familial Mediterranean fever (FMF) has traditionally been considered an autosomal-rece

30 Familial Mediterranean fever (FMF) is a recessive disorder characterized by episodes o

31 Familial Mediterranean fever (FMF) is a recessive disorder characterized by episodes o

32 Familial Mediterranean fever (FMF) is a recessive disorder characterized by episodes o

33 Familial Mediterranean fever (FMF) is a recessive disorder of inflammation caused by m

34 Familial Mediterranean fever (FMF) is a recessively inherited autoinflammatory disorde

35 Familial Mediterranean fever (FMF) is a recessively inherited disorder characterized b

36 Familial Mediterranean fever (FMF) is an archetypal autoinflammatory disorder, which i

37 Familial Mediterranean fever (FMF) is an autoinflammatory disease caused by mutations

38 Familial Mediterranean fever (FMF) is an autosomal recessive disease due to mutations

39 Familial Mediterranean fever (FMF) is an IL-1beta-dependent autoinflammatory disease c

40 Familial Mediterranean Fever (FMF) is an inherited autoinflammatory disorder character

41 Familial Mediterranean fever (FMF) is an inherited disorder characterized by recurrent

42 f serositis in familial Mediterranean fever (FMF) is not completely understood.

43 Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disea

44 urrounding the familial Mediterranean fever (FMF) locus.

45 In familial Mediterranean fever (FMF) MEFV mutations lead to gain of pyrin function, resu

46 patients with familial Mediterranean fever (FMF) that is resistant to or intolerant of colchicine.

47 patients with familial Mediterranean fever (FMF) who reach end-stage renal disease (ESRD) due to rea

48 ate region for familial Mediterranean fever (FMF), a recessively inherited disorder of inflammation l

49 e gene causing familial Mediterranean fever (FMF), a recessively inherited disorder of inflammation.

50 of pyrin cause familial Mediterranean fever (FMF), the most common Mendelian autoinflammatory disease

51 g the gene for familial Mediterranean fever (FMF), we identified a number of transcripts from a 275-k

52 atory disorder Familial Mediterranean Fever (FMF).

53 utations cause Familial Mediterranean Fever (FMF).

54 Familial Mediterranean fever (FMF; MIM 249100) is an autosomal recessive disease chara

55 on multiplexing (SDM) using few-mode fibers (FMF) has been proposed and demonstrated.

56 14 periodontally healthy patients with FMF (FMF-HC).

57 A candidate gene for FMF was identified by positional cloning and named "MEFV

58 The gene responsible for FMF--MEFV--has been identified and its role in inflammat

59 been independently identified by the French FMF Consortium, and the others are novel.

60 e substantially increased in leukocytes from FMF patients compared with healthy controls.

61 ents with folliculotropic mycosis fungoides (FMF) have a worse prognosis than patients with classic m

62 Pyrin variants harboring FMF-associated B30.2 mutations were cleaved more efficie

63 n 46 patients diagnosed clinically as having FMF and carrying only 1 high-penetrance FMF mutation.

64 s in the inflammasome adaptor Pyrin, but how FMF mutations alter signaling in FMF patients is unknown

65 n and those with a double mutation; however, FMF patients of both types showed higher protein express

66 v(V726A/V726A)) was generated to model human FMF.

67 mation similar to but more severe than human FMF.

68 The possibility of prevention of attacks in FMF needs to be tested through stress-reduction interven

69 e unique therapeutic effect of colchicine in FMF may be dependent on this interaction.

70 eukin-1 is a key proinflammatory cytokine in FMF.

71 uding 5 newly identified microsatellites, in FMF families.

72 Pyrin, the protein mutated in FMF, regulates caspase-1 activation and consequently IL-

73 ysaccharide alone, which is also observed in FMF patients.

74 in, but how FMF mutations alter signaling in FMF patients is unknown.

75 hat are substantially higher in HIDS than in FMF.

76 ived from the PBMC of patients with inactive FMF.

77 d as an unusual case of dominantly inherited FMF.

78 nts with colchicine-resistant or -intolerant FMF.

79 transcribed sequences isolated from a 315-kb FMF central region (between D16S468/D16S3070 and cosmid

80 data were distinguished: early skin-limited FMF (group A; n = 84; 5-year and 10-year OS, 92% and 72%

81 year OS, 92% and 72%); advanced skin-limited FMF (group B; n = 102; 5-year and 10-year OS, 55% and 28

82 d polarization entanglement over a 1-km-long FMF.

83 ssion as compared with controls and with non-FMF patients with active inflammation.

84 iscuss the clinical and molecular aspects of FMF in Arabs.

85 count for a large percentage of the cases of FMF and suggest that some of these mutations arose befor

86 fic IL-1 cytokine instigating development of FMF and the enzymatic caspase involved in its secretion

87 ce of clinical symptoms for the diagnosis of FMF and the initiation of a trial of colchicine.

88 the synchronous inflammatory environment of FMF attack leads to intracellular production of IL-1beta

89 Differences in the frequency of FMF attacks and adverse events between rilonacept and pl

90 Rilonacept reduces the frequency of FMF attacks and seems to be a treatment option for patie

91 Small sample size, heterogeneity of FMF mutations, age, and participant indication (colchici

92 -fat diet did not increase the likelihood of FMF attacks.

93 ted IL-1beta release in the manifestation of FMF.

94 ted upon microtubule disassembly in PBMCs of FMF patients but not in cells of patients afflicted with

95 g FMF and enables immunological screening of FMF mutations.

96 F and confirm the existence of a subgroup of FMF with a favorable prognosis.

97 ore the existence of a significant subset of FMF patients who are carriers of only 1 MEFV mutation an

98 ving FMF and carrying only 1 high-penetrance FMF mutation.

99 Multiple stressful life events predicted FMF attacks 2 days following the event.

100 associated with the transmission of a second FMF allele.

101 Distinction between early plaque-stage FMF and advanced plaque-stage FMF was made by a blinded

102 ients with histologically early plaque-stage FMF had a very similar overall survival (OS) rate to pat

103 ts with histologically advanced plaque-stage FMF was almost identical to that of patients presenting

104 y plaque-stage FMF and advanced plaque-stage FMF was made by a blinded review of skin biopsy specimen

105 wnstream of Pyrin dephosphorylation and that FMF mutations enable microtubule-independent assembly of

106 The FMF gene (MEFV) encodes a novel 781 amino acid protein;

107 The FMF gene (MEFV) was cloned recently, and four missense m

108 The FMF gene, MEFV, was recently identified by positional cl

109 y founder haplotype analysis, to contain the FMF gene.

110 ning approximately 1050 kb that contains the FMF critical region.

111 en two major developments in this field: the FMF gene was identified on chromosome 16p by positional

112 rum-TOS and serum-OSI levels of those in the FMF-CP and CP groups at baseline and 6 weeks (P >0.05).

113 The GCF-TOS level in the FMF-CP group was significantly higher than that in the F

114 Serum-TAS levels in the FMF-CP group were significantly higher than those in the

115 up was significantly higher than that in the FMF-HC group at baseline and 6 weeks.

116 ucted a 1-Mb cosmid contig that includes the FMF critical region.

117 only has permitted the identification of the FMF gene (MEFV), but also has provided us an opportunity

118 were significantly reduced in members of the FMF-CP group (P <0.05).

119 The GCF-TAS levels in members of the FMF-CP group were significantly higher than those of mem

120 These methods place the FMF susceptibility gene within 0.305 cM of D16S246 (2-LO

121 mately 700 kb of genomic DNA surrounding the FMF locus was assembled.

122 nd ZNF210 are 10 and 120 kb telomeric to the FMF gene, respectively.

123 tly higher in the CP group compared with the FMF-CP group (P <0.05).

124 iods, and high-fat food consumption prior to FMF attacks and on attack-free random days.

125 The binding of 14-3-3 and PKN proteins to FMF-associated mutant pyrin was substantially decreased,

126 a truncated pyrin molecule that, similar to FMF patients, retains the full PYRIN domain.

127 des a conceptual framework for understanding FMF and enables immunological screening of FMF mutations

128 We observed a unique FMF haplotype common to Iraqi Jews, Arabs, and Armenians

129 ever, it remains controversial as to whether FMF is due to the loss of an inhibitor of inflammation o

130 116.6 +/- 67.5 months 11 patients (55%) with FMF died versus 26 patients (31%) in the control group.

131 f perceived stress were also associated with FMF attacks.

132 at D16S246 was significantly associated with FMF in Moroccan and non-Moroccan Jews but not in Armenia

133 te phase response seen in HIDS compared with FMF reflects greater production of acute phase protein-i

134 a high degree of linkage disequilibrium with FMF, was sequenced to completion, and the sequence annot

135 essed acute-phase proteins in a patient with FMF and amyloidosis.

136 , and 14 periodontally healthy patients with FMF (FMF-HC).

137 The outcomes of 20 patients with FMF and biopsy-proven AA amyloidosis that were transplan

138 h Italian and Ashkenazi Jewish patients with FMF and other affected populations.

139 Most patients with FMF carry missense mutations in the C-terminal half of t

140 Neutrophils from patients with FMF during remission are resistant to autophagy-mediated

141 Overall, 220 patients with FMF had been registered, but 17 patients with incomplete

142 MB) staging system to classify patients with FMF in a clinically meaningful way.

143 peripheral neutrophils from 3 patients with FMF isolated both during attacks and remission, 8 patien

144 ral blood mononuclear cells of patients with FMF or HIDS was attenuated by activation of PKN1 and PKN

145 Median time of death for patients with FMF was 61 months (range, 16-81) after transplantation.

146 tudies described a subgroup of patients with FMF with a more favorable prognosis.

147 Data were obtained from 13 patients with FMF with generalized CP (FMF-CP), 15 systemically health

148 ctive clinical picture in Arab patients with FMF, and the range and distribution of MEFV mutations is

149 d TNF-alpha than did PBMC from patients with FMF, but unstimulated PBMC from the latter group release

150 cohort study, we followed 203 patients with FMF, included in the Dutch Cutaneous Lymphoma Registry b

151 5%, and 39%, respectively, for patients with FMF, versus 84%, 68% and 63%, respectively, for the cont

152 of the present study show that patients with FMF-CP displayed reduced oxidative stress and increased

153 majority of mutations found in patients with FMF.

154 se of death in the majority of patients with FMF.

155 ion of inflammatory attacks in patients with FMF.

156 extracellular traps (NETs) in patients with FMF.