ライフサイエンスコーパス: CGD

1 CGD caused by A22(0) and A67(0) subtypes manifests as se

2 CGD is a severe immunodeficiency caused by defects in th

3 CGD mice, lacking a respiratory burst, developed accentu

4 CGD results from defective production of reactive oxygen

5 ese specific bands, specimens from 79 of 175 CGD patients (45.1%) and 23 of 93 healthy donors (24.7%)

6 tients with CGD and control subjects (14.6%, CGD; 6.3%, controls; P=0.39).

7 A CGD isolate, CGD1, showed higher cell association than t

8 it a compound phenotype consisting of both a CGD-like immune defect and a balance disorder caused by

9 in culture-confirmed cases and 5 additional CGD patients.

10 Adult CGD patients displayed similar characteristics and rates

11 bacter immunoreactivity is more common among CGD patients and, perhaps, among healthy donors than was

12 servoir(s), and incidence of infection among CGD patients and the general population are unknown.

13 ociation between donor chronological age and CGD (P=0.018).

14 ociation between donor chronological age and CGD (P=0.018).

15 been shown to be at greater risk for AR and CGD, this does not appear to be associated with shorter

16 been shown to be at greater risk for AR and CGD, this does not appear to be associated with shorter

17 91(phox) expression in patients with CGD and CGD carriers might affect the B-cell compartment and mai

18 limiting step in tryptophan degradation, and CGD patients do not appear to have either hematopoietic

19 st defense pathway intact in both normal and CGD MDM, or whether it occupies a distinct intracellular

20 racteristics were similar in both normal and CGD MDM.

21 eased during phagocytosis by both normal and CGD PMN demonstrating responses to oxygen-independent PM

22 for bacterial resistance to both normal and CGD PMN.

23 aneously, in G. bethesdensis with normal and CGD PMN.

24 hagocytosis of G. bethesdensis by normal and CGD polymorphonuclear leukocytes (CGD PMN) required heat

25 Cells from both normal and CGD subjects internalized G. bethesdensis similarly.

26 n PMN from healthy subjects (normal PMN) and CGD PMN during incubation with G. bethesdensis and, simu

27 Wild-type, PXR-null (PXR-/-), and CGD-sensitive C57L mice were placed on a lithogenic diet

28 ID, and enrollment in the studies on WAS and CGD is underway.

29 re significantly higher than those in WT and CGD mice after challenge with S. aureus.

30 Similar findings were observed with another CGD pathogen, Serratia marcescens, but not with Escheric

31 H) oxidase leads to autosomal recessive (AR) CGD.

32 formed TL in donor or recipient DNA with AR, CGD, or graft failure, although we did observe an associ

33 formed TL in donor or recipient DNA with AR, CGD, or graft failure, although we did observe an associ

34 atients with XL-CGD than in patients with AR-CGD (relative risk, 2.22; 95% CI, 1.43-3.46).

35 flammatory episodes than do patients with AR-CGD.

36 08 in patients with autosomal-recessive [AR] CGD).

37 he clinical host tropism of G. bethesdensis, CGD PMN were unable to kill this organism, while normal

38 (efferocytosis) of apoptotic neutrophils by CGD macrophages may contribute to this effect.

39 tic neutrophils and enhanced phagocytosis by CGD Ms.

40 reduced circulating CD27(+) memory B cells, CGD patients maintain an intact humoral immunologic memo

41 pothesized that inflammasomes detect certain CGD pathogens upstream of neutrophil killing.

42 s by Staphylococcus xylosus or by the common CGD pathogen Staphylococcus aureus.

43 2), malignancy (n=2), HIV (n=1), concomitant CGD and DM (n=1), and steroid therapy for nephrotic synd

44 e LXR transgenic mice may offer a convenient CGD model to develop therapeutic interventions for this

45 The Candida Genome Database (CGD) is a freely available online resource that provides

46 The Candida Genome Database (CGD) provides online access to genomic sequence data and

47 d constructed the Clinical Genomic Database (CGD), a searchable, freely Web-accessible database of co

48 tus in a murine X-linked gp91phox-deficiency CGD model.

49 Cholesterol gallstone disease (CGD) results from a biochemical imbalance of lipids and

50 and possibly cholesterol gallstone disease (CGD).

51 on, including chronic granulomatous disease (CGD) (n=4), diabetes mellitus (DM) (n=2), malignancy (n=

52 ients without chronic granulomatous disease (CGD) achieved an OS at 3 years of 88.9% (n = 18), compar

53 physiology of chronic granulomatous disease (CGD) and sepsis.

54 patients with chronic granulomatous disease (CGD) and those with HIV infection.

55 abscesses in chronic granulomatous disease (CGD) are typically difficult to treat and often require

56 Chronic granulomatous disease (CGD) can be cured by allogeneic hemopoietic stem cell tr

57 patients with chronic granulomatous disease (CGD) cause susceptibility to extracellular and intracell

58 Patients with chronic granulomatous disease (CGD) experience immunodeficiency because of defects in t

59 patients with chronic granulomatous disease (CGD) fail to produce microbicidal concentrations of reac

60 patients with chronic granulomatous disease (CGD) for reasons and consequences that remain unclear.

61 prognosis of Chronic Granulomatous Disease (CGD) has greatly improved, few studies have focused on i

62 Patients with chronic granulomatous disease (CGD) have a mutated NADPH complex resulting in defective

63 l features of chronic granulomatous disease (CGD) in 1959.

64 mbles that of chronic granulomatous disease (CGD) in extent and features of colonic inflammation obse

65 Chronic granulomatous disease (CGD) is a primary immune deficiency characterized by a d

66 Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by defective p

67 Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by seri

68 Chronic granulomatous disease (CGD) is a rare genetic disorder, predisposing affected i

69 Chronic granulomatous disease (CGD) is a rare phagocytic disorder that results in not o

70 Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder of phag

71 Chronic granulomatous disease (CGD) is an inherited disorder characterized by recurrent

72 patients with chronic granulomatous disease (CGD) is Aspergillus fumigatus followed by A. nidulans; o

73 Chronic granulomatous disease (CGD) is associated with significant morbidity and mortal

74 Chronic granulomatous disease (CGD) is characterized by overexuberant inflammation and

75 Chronic granulomatous disease (CGD) is characterized by recurrent life-threatening bact

76 Chronic granulomatous disease (CGD) is due to defective nicotinamide adenine dinucleoti

77 deficiency in chronic granulomatous disease (CGD) is well characterized.

78 x2)-deficient chronic granulomatous disease (CGD) mice that lack the gp91(phox) (gp91(phox-/-)) catal

79 isolated from chronic granulomatous disease (CGD) patients and mice, formed more frequent multiple ps

80 Chronic granulomatous disease (CGD) patients are susceptible to life-threatening infect

81 Chronic granulomatous disease (CGD) patients have recurrent life-threatening bacterial

82 e predisposes chronic granulomatous disease (CGD) patients to infection, and also to unexplained, exa

83 patients with chronic granulomatous disease (CGD) results in susceptibility to certain pathogens seco

84 patients with chronic granulomatous disease (CGD), a deficiency in the phagocyte NADPH oxidase.

85 uffering from chronic granulomatous disease (CGD), a primary immunodeficiency caused by a defect in t

86 unction cause chronic granulomatous disease (CGD), a primary immunodeficiency characterized by dysfun

87 patients with chronic granulomatous disease (CGD), a primary immunodeficiency marked by a defect in N

88 hox) leads to chronic granulomatous disease (CGD), a severe immune disorder characterized by the inab

89 Chronic granulomatous disease (CGD), an immunodeficiency with recurrent pyogenic infect

90 patients with chronic granulomatous disease (CGD), and others.

91 nzyme lead to chronic granulomatous disease (CGD), associated with increased susceptibility to both p

92 treatment for chronic granulomatous disease (CGD), but the safety and efficacy of HSCT from unrelated

93 In chronic granulomatous disease (CGD), defective phagocytic nicotinamide adenine dinucleo

94 atus-infected chronic granulomatous disease (CGD), hydrocortisone-treated, and neutropenic mice.

95 sociated with chronic granulomatous disease (CGD), which is characterized by recurrent and life-threa

96 patients with chronic granulomatous disease (CGD).

97 patients with chronic granulomatous disease (CGD).

98 patients with chronic granulomatous disease (CGD).

99 osis (CF) and chronic granulomatous disease (CGD).

100 unodeficiency chronic granulomatous disease (CGD).

101 unodeficiency chronic granulomatous disease (CGD).

102 me (WAS), and chronic granulomatous disease (CGD).

103 ADPH oxidase (chronic granulomatous disease [CGD]) and corticosteroid-induced immunosupression lead t

104 receptor gamma (PPARgamma) activation during CGD inflammation is deficient, leading to altered macrop

105 e rejection (AR), chronic graft dysfunction (CGD), and graft failure of kidney allografts.

106 e rejection (AR), chronic graft dysfunction (CGD), and graft failure of kidney allografts.

107 Eighty CGD patients (71 males [88.7%], 59 X-linked [73.7%], med

108 reby contribute to resolution of exaggerated CGD inflammation.

109 to previous NAR Database articles featuring CGD, we describe a new tab that we have added to the Loc

110 The first CGD gene therapy trial resulted in only short-term marki

111 For CGD, equivalent outcomes can be obtained with MRD or MUD

112 s of 88.9% (n = 18), compared with 81.8% for CGD patients (n = 11).

113 d on a lithogenic diet and then analyzed for CGD at the biochemical, histological, and gene-regulatio

114 ese data open new therapeutic approaches for CGD-related inflammatory manifestations.

115 h, PPARgamma may be a therapeutic target for CGD, and possibly other inflammatory conditions where ab

116 may be developed as a therapeutic target for CGD.

117 orans strains, 2 from CF patients and 2 from CGD patients.

118 ssociated significantly more with cells from CGD patients than with those from healthy donors.

119 We used leukocytes from CGD and healthy donors and compared cell association, in

120 trate that efferocytosis by macrophages from CGD (gp91(phox)(-/-)) mice was suppressed ex vivo and in

121 Here, we demonstrate that macrophages from CGD mice and blood monocytes from CGD patients display m

122 hages from CGD mice and blood monocytes from CGD patients display minimal recruitment of microtubule-

123 to 51% (P < 0.01) in vitro in monocytes from CGD patients.

124 Diverse M populations from CGD (gp91(phox)(-/-)) and wild-type mice, as well as hum

125 lthy subjects, whereas in patients with HIV, CGD, and Crohn disease, there was a significant increase

126 rom gp91(PHOX)- or p47(PHOX)-deficient human CGD donors.

127 gp91(phox-/-) mice, a murine model of human CGD, would enhance phagocyte oxidant production and kill

128 considered a deficiency of innate immunity, CGD is also linked to dysfunctional T cell reactivity.

129 m Ab can be protective in an immunodeficient CGD host.

130 of Staphylococcus aureus liver abscesses in CGD.

131 that PPARgamma expression and activation in CGD macrophages were significantly deficient at baseline

132 e activation, anakinra restored autophagy in CGD mice in vivo, with increased Aspergillus-induced LC3

133 ce impaired engulfment of apoptotic cells in CGD.

134 which are linked pathological conditions in CGD that can be restored by IL-1 receptor blockade.

135 ne cells, which incidentally is defective in CGD patients, is considered to be a fundamental process

136 ction and partially restores host defense in CGD.

137 hypothesized that impaired efferocytosis in CGD due to macrophage skewing contributes to enhanced in

138 B-cell responses, as measured by Elispot in CGD patients compared with HDs.

139 against S. aureus, but not A. fumigatus, in CGD mice.

140 27(-) to CD27(+) was significantly higher in CGD patients compared with HDs.

141 way that contributes to hyperinflammation in CGD and in septic patients.

142 proposed as a cause of hyperinflammation in CGD and this pathway has been considered for clinical in

143 we review two clinical cases of fatal IA in CGD patients and describe a new etiologic agent of IA re

144 This is the first report documenting IA in CGD patients caused by a species belonging to the Asperg

145 n addition to correcting immunodeficiency in CGD, IFN-gamma priming of Ms restores clearance of apopt

146 ation during peritonitis and was impaired in CGD mice (versus wild-type), leading to accumulation of

147 ole MIC and causes more chronic infection in CGD mice than A. fumigatus.

148 nd the impact of common severe infections in CGD, we examined the records of 268 patients followed at

149 r driver of enhanced sterile inflammation in CGD in the response to damaged cells.

150 Thus, inflammation in CGD is due to IL-1-dependent mechanisms, such as decreas

151 underlying cause of enhanced inflammation in CGD.

152 Liver involvement in CGD includes vascular abnormalities, which may lead to n

153 a did not enhance G. bethesdensis killing in CGD monocytes, it restricted growth in proportion to CGD

154 ulans is the second most encountered mold in CGD patients, causing almost exclusively invasive infect

155 t occupies a distinct intracellular niche in CGD MDM, we assessed the trafficking patterns of this or

156 pathway, the pathogenesis of A. nidulans in CGD cannot be explained.

157 bmGT was persistently detected only in CGD lungs.

158 lderia cepacia, an opportunistic pathogen in CGD patients, whereas MN(-) mice died.

159 sis is an emerging Gram-negative pathogen in CGD that resists killing by PMN of CGD patients (CGD PMN

160 and normalized the course of peritonitis in CGD mice.

161 er these processes are mutually regulated in CGD and whether defective autophagy is clinically releva

162 ites were elevated rather than suppressed in CGD donors.

163 Survival in CGD has increased over the years, but infections are sti

164 cirrhotic portal hypertension on survival in CGD, all records from 194 patients followed up at the Na

165 ns remain a major determinant of survival in CGD.

166 est that OLFM4 may be an important target in CGD patients for the augmentation of host defense agains

167 for clearance, and can persist long-term in CGD mononuclear phagocytes, most likely relating to the

168 R sensitized mice to lithogenic diet-induced CGD, characterized by decreases in biliary concentration

169 ersistence of this microorganism in infected CGD patients.

170 Here, we report the incorporation into CGD of Assembly 22, the first chromosome-level, phased d

171 e describe the incorporation of JBrowse into CGD, which allows online browsing of mapped high through

172 normal and CGD polymorphonuclear leukocytes (CGD PMN) required heat-labile serum components (e.g., C3

173 Mutations in CYBB cause X-linked CGD and account for 65% to 70% of cases in Western count

174 manifestations associated with the X-linked CGD carrier state.

175 strongly predicts infection risk in X-linked CGD carriers, and the carrier state itself is associated

176 g the gp91(phox-/-) murine model of X-linked CGD in a well-characterized model of sterile, zymosan-in

177 We describe 9 X-linked CGD patients with staphylococcal liver abscesses refract

178 than 20% marking in 2 patients with X-linked CGD.

179 To answer this need, CGD has expanded beyond storing data solely for C. albic

180 nts with infectious (HIV) and noninfectious (CGD and Crohn disease) diseases that have been associate

181 ivation with interleukin 4 (IL-4) normalized CGD macrophage efferocytosis, whereas classical activati

182 Normal but not CGD monocytes and MDM killed G. bethesdensis and require

183 ance genes in the presence of normal but not CGD PMN.

184 mice; neither was detected in serum/BALF of CGD or steroid-treated mice.

185 7(+) but also IgG(+) B cells in the blood of CGD patients compared with healthy donors (HDs).

186 cytes and neutrophils purified from blood of CGD patients, who have deficient Nox2 activity.

187 nal memory B cells, CD27(-)IgG(+) B cells of CGD patients expressed activation markers and had underg

188 es that defined the cellular deficiencies of CGD, specifically finding that improper degranulation of

189 All patients had confirmed diagnoses of CGD, and genotype was determined where possible.

190 We studied 15 patients given a diagnosis of CGD and followed in our institution.

191 Although the effect of CGD mainly reflects on the phagocytic compartment, B-cel

192 The goal of CGD is to facilitate and accelerate research into Candid

193 Infection of CGD mice with G. bethesdensis confirmed acquisition of h

194 also a significant clinical manifestation of CGD.

195 The mission of CGD is to facilitate and accelerate research into Candid

196 agocyte oxidase p47(phox)-deficient model of CGD and found that UV-inactivated Streptococcus pneumoni

197 of either T cell subset in a mouse model of CGD is contingent upon the nature of the fungal vaccine,

198 In a mouse model of CGD, strain CGD1 was virulent while Env1 was avirulent.

199 receptor (PXR) has a role in pathogenesis of CGD.

200 ctions and shed light on the pathogenesis of CGD.

201 thogen in CGD that resists killing by PMN of CGD patients (CGD PMN) and inhibits PMN apoptosis throug

202 We document the reversion of CGD inflammatory status by the mammalian target of rapam

203 studied the clinical course and sequelae of CGD patients diagnosed before age 16, at various adult t

204 of invasive aspergillosis in the setting of CGD and corticosteroid-induced immunosupression.

205 potent autophagy inducer, in the setting of CGD.

206 (phox)- and gp91(phox)-deficient subtypes of CGD and independent of risk factors in multivariate regr

207 y shed light on the unique susceptibility of CGD patients to specific pathogens.

208 Treatment of CGD mice with IFN-gamma also enhanced uptake of apoptoti

209 Data on CGD carriers reveal that such alterations are related to

210 njection of PS (whose exposure is lacking on CGD apoptotic neutrophils) in vivo restored IL-4-depende

211 OS production, reproducing the pathognomonic CGD cellular phenotype.

212 that resists killing by PMN of CGD patients (CGD PMN) and inhibits PMN apoptosis through unknown mech

213 episodes of infection/admission/surgery per CGD life year (95% CI, 0.69-0.75 events per year).

214 s of Granulibacter in cells from permissive (CGD) and nonpermissive (normal) hosts and identifies pot

215 , whereas p47(phox)-deficient (p47(phox-/-)) CGD mice show survival rates that are similar to those o

216 PXR prevented CGD via its coordinate regulation of the biosynthesis an

217 IF stabilization within the mucosa protected CGD mice from severe colitis.

218 tment and T helper 17 responses and protects CGD mice from colitis and also from invasive aspergillos

219 oding gp91(phox) leads to X-linked recessive CGD.

220 G. bethesdensis resists killing by serum, CGD polymorphonuclear leukocytes (PMN), and antimicrobia

221 nters have partnered with the PIDTC to study CGD.

222 The CGD currently includes a total of 2,616 genes organized

223 The CGD Web site is organized around Locus pages, which disp

224 The CGD will be regularly maintained and updated to keep pac

225 For each entry, the CGD includes the gene symbol, conditions, allelic condit

226 Eventually, the CGD may assist the rapid curation of individual genomes

227 sive infections caused by A. nidulans in the CGD patient and is intended to direct further research b

228 sis and optimize new data incorporation, the CGD also includes all genetic conditions for which genet

229 pecific antibodies were also normal in these CGD patients.

230 ng in their disintegration may contribute to CGD inflammation.

231 cytes, it restricted growth in proportion to CGD PMN residual superoxide production, providing a pote

232 the in vitro results, adoptively transferred CGD murine neutrophils showed impaired in vivo recruitme

233 In IFN-gamma-treated CGD MDM, G. bethesdensis persisted for the duration of t

234 e of these findings was assessed by treating CGD patients who had severe colitis with IL-1 receptor b

235 GT levels were higher in neutropenic versus CGD or steroid-treated lungs.

236 nflammation and autoimmunity associated with CGD.

237 In contrast, carriers with CGD-type infections had median %DHR(+) values of 8% (n =

238 can be used safely in HSCT for children with CGD and high-risk clinical features, achieving excellent

239 Children with CGD not undergoing transplantation have more serious inf

240 nd Olfm4 double-deficient mice compared with CGD mice.

241 up at the National Institutes of Health with CGD were reviewed.

242 hanisms are dysregulated in individuals with CGD have not been determined.

243 gal CD8(+) T cell memory in individuals with CGD.

244 CD8(+) T cells failed to occur in mice with CGD due to defective DC endosomal alkalinization and aut

245 lved, making the management of patients with CGD a complex, multidisciplinary task.

246 We compared patients with CGD according to the severity of NADPH oxidase deficienc

247 icated in human monocytes from patients with CGD after ex vivo pioglitazone treatment.

248 tcomes and overall survival in patients with CGD after HSCT.

249 id and coronary arteries of 41 patients with CGD and 25 healthy controls in the same age range.

250 ctive gp91(phox) expression in patients with CGD and CGD carriers might affect the B-cell compartment

251 cification was similar between patients with CGD and control subjects (14.6%, CGD; 6.3%, controls; P=

252 tifungal activity of PMNs from patients with CGD at a significantly lower concentration, compared wit

253 tor 9 pathways were reduced in patients with CGD compared with those seen in age-matched healthy cont

254 nary artery atherosclerosis in patients with CGD despite the high prevalence of traditional risk fact

255 ave shown that phagocytes from patients with CGD display a defect in autophagy and a reactive oxygen

256 regulation in phagocytes among patients with CGD during fungal pathogenesis, we evaluated the effect

257 report here the results of 89 patients with CGD from 73 Turkish families in a multicenter study.

258 unctional, and genetic data of patients with CGD from Turkey.

259 Despite this, patients with CGD had a 22% lower internal carotid artery wall volume

260 Patients with CGD had significant elevations in traditional risk facto

261 died the B-cell compartment of patients with CGD in terms of phenotype and ability to produce reactiv

262 istent chronic inflammation in patients with CGD is associated with hematopoietic proliferative stres

263 hese data suggest mortality in patients with CGD is associated with the development of noncirrhotic p

264 hagy is clinically relevant in patients with CGD is unknown.

265 We show that patients with CGD present a consistent inflammatory phenotype defined

266 ogether, our data suggest that patients with CGD present a defective B-cell compartment in terms of f

267 Patients with CGD suffer from severe, life-threatening infections and

268 compartment is impaired among patients with CGD, as indicated by reduced total (CD19(+)CD27(+)) and

269 flammatory state of PBMCs from patients with CGD, as observed by decreased tumor necrosis factor alph

270 nearly absent in monocytes of patients with CGD.

271 e of invasive aspergillosis in patients with CGD.

272 nist anakinra in phagocytes of patients with CGD.

273 itioning in HSCT for pediatric patients with CGD.

274 e hematopoietic compartment in patients with CGD.

275 responses are also impaired in patients with CGD.

276 ents with sepsis, particularly in those with CGD.

277 dase subunits, activation of iNKT cells by X-CGD peritoneal exudate macrophages was impaired during s

278 nd X-linked chronic granulomatous disease (X-CGD) mice.

279 th X-linked chronic granulomatous disease (X-CGD) that lack oxidase subunits, activation of iNKT cell

280 th X-linked chronic granulomatous disease (X-CGD), a defect of neutrophil microbicidal reactive oxyge

281 th X-linked chronic granulomatous disease (X-CGD), caused by mutations in the gp91phox subunit of the

282 th X-linked chronic granulomatous disease (X-CGD).

283 uch, we initiated a gene therapy trial for X-CGD to treat severe infections unresponsive to conventio

284 hat mature neutrophils differentiated from X-CGD iPSCs lack ROS production, reproducing the pathognom

285 allele of the "safe harbor" AAVS1 locus in X-CGD iPSCs without off-target inserts resulted in sustain

286 d LD-IR was also effective conditioning in X-CGD mice for engraftment of X-CGD donor HSCs transduced

287 ed neutrophilic peritoneal inflammation in X-CGD mice.

288 ion of otherwise prolonged inflammation in X-CGD.

289 ited by tissue injury, X-linked Cybb-null (X-CGD) mice exhibited increased release of IL-1alpha and I

290 ditioning in X-CGD mice for engraftment of X-CGD donor HSCs transduced ex vivo with a lentiviral vect

291 targeting may be applied to correction of X-CGD using zinc finger nuclease and patient iPSCs.

292 ibition of cytokine signaling in mice with X-CGD reduced HPC numbers but had only minor effects on th

293 e marrow cells from patients and mice with X-CGD revealed a dysregulated hematopoiesis characterized

294 In mice with X-CGD, increased cycling of HSCs, expansion of HPCs, and i

295 In patients with X-CGD, there was a clear reduction in the proportion of HS

296 imicking the effects observed in mice with X-CGD.

297 therapy trials targeting ADA-SCID, SCID-X1, CGD and WAS, review the pitfalls, and outline the recent

298 Patients with XL-CGD have a higher risk of developing inflammatory episod

299 isodes was 2-fold higher in patients with XL-CGD than in patients with AR-CGD (relative risk, 2.22; 9

300 on-year (0.18 in patients with X-linked [XL] CGD and 0.08 in patients with autosomal-recessive [AR] C