ライフサイエンスコーパス: immune deficiency

1 enhance T-cell survival (eg, vaccination and immune deficiency).

2 (ICU) patients, even in those without prior immune deficiency.

3 stemic diseases and exhibited no evidence of immune deficiency.

4 have been associated with inherited forms of immune deficiency.

5 lammatory autoimmune disease and diseases of immune deficiency.

6 type, which may contribute to age-associated immune deficiency.

7 preventing and/or correcting post-BMT T cell immune deficiency.

8 ral anti-SIV responses, and show no signs of immune deficiency.

9 identify a novel mechanism of age-associated immune deficiency.

10 manifested by both B-cell autoreactivity and immune deficiency.

11 nderstanding the pathogenesis of progressive immune deficiency.

12 ific defects in NEMO ubiquitination and thus immune deficiency.

13 an important complication in the context of immune deficiency.

14 ons of cell networks, leading to generalized immune deficiency.

15 Wnt pathway in normal B cell function and FA immune deficiency.

16 in the different segments of the eye due to immune deficiency.

17 , SCFA or dietary fiber intake restores this immune deficiency.

18 nsplantation, and 10% died mostly because of immune deficiency.

19 erturbed by single-gene mutations in primary immune deficiency.

20 toimmunity, lymphoproliferation, and humoral immune deficiency.

21 gain-of-function mutations may also promote immune deficiency.

22 2 unrelated families in association with an immune deficiency.

23 catalytic subunit, are capable of promoting immune deficiency.

24 fic associations of autoimmune diseases with immune deficiencies.

25 results in various lymphoid malignancies and immune deficiencies.

26 s and monitoring of patients with cancer and immune deficiencies.

27 in young foals and in people with underlying immune deficiencies.

28 s with a variety of debilitating diseases or immune deficiencies.

29 nding of the molecular basis of many primary immune deficiencies.

30 sed to investigate the cell biology of human immune deficiencies.

31 n, a common disturbance of atypical clinical immune deficiencies.

32 the context of AIDS and other CD4(+) T cell immune deficiencies.

33 interactor (TACI) result in common variable immune deficiency, a syndrome marked by recurrent infect

34 and senescent T cells, lymphadenopathy, and immune deficiency (activated PI3Kdelta syndrome [APDS]).

35 denosine deaminase-deficient severe combined immune deficiency (ADA-SCID) can provide significant lon

36 denosine deaminase-deficient severe combined immune deficiency (ADA-SCID) was enrolled in a study of

37 opic MLV in B6 mice with a range of distinct immune deficiencies affecting antibody production.

38 te adhesion deficiency are the major primary immune deficiencies affecting phagocytic blood cells.

39 peutic target in several genetic diseases of immune deficiency affecting cytokine signaling.

40 r the rapid and durable correction of T-cell immune deficiency after BMT, and the induction of tolera

41 se data implicate AD as a cause of secondary immune deficiency after SCI and reveal novel therapeutic

42 the complex relationship between the genetic immune deficiencies and autoimmunity.

43 to conceptualize the association of primary immune deficiencies and autoimmunity.

44 itioning regimen can be used to overcome the immune deficiencies and prevent the malignancies observe

45 mune degradation ultimately leading to overt immune deficiency and AIDS.

46 defects, KS is also characterized by humoral immune deficiency and autoimmune disease, yet no detaile

47 Immune deficiency and autoimmunity have been recognized

48 s, but defective inside-out signaling causes immune deficiency and bleeding problems.

49 deaminase-deficient forms of severe combined immune deficiency and chronic granulomatous disease.

50 Pre-ART immune deficiency and early immune recovery may contribu

51 orphic alleles typically cause a syndrome of immune deficiency and ectodermal dysplasia.

52 ineous families with childhood-onset humoral immune deficiency and features of autoimmunity shared ge

53 especially those with delayed-onset combined immune deficiency and granulomatous/autoimmune manifesta

54 Although the determinants of immune deficiency and immune restoration in chronic huma

55 tion limit nutrients and are associated with immune deficiency and increased susceptibility to infect

56 A 57-year-old woman with common variable immune deficiency and liver failure of unknown etiology

57 ulation of these proteins is associated with immune deficiency and metastasis [1-4].

58 er formation in all strains, causing humoral immune deficiency and susceptibility to infection.

59 l B-cell differentiation, leading to humoral immune deficiency and, in some cases, autoimmunity.

60 rated antiviral roles of the Drosophila Imd (immune deficiency) and Jak-STAT innate immunity pathways

61 ell-based therapies for cancer, HIV, primary immune deficiencies, and autoimmune diseases, but geneti

62 ukemia and lymphoma, patients with inherited immune deficiencies, and patients on immunosuppressive t

63 ity, hypersensitivity to ionizing radiation, immune deficiency, and sterility [1, 2]-phenotypes that

64 thymic epithelial cell (TEC) injury, T-cell immune deficiency, and susceptibility to opportunistic i

65 that lack of TNFR1 leads to a skin-specific immune deficiency, and that resident skin cells have a c

66 cy of PNP in humans causes a specific T-cell immune deficiency, and transition state analogue inhibit

67 ily affected by early-onset IBD, progressive immune deficiency, and, in some cases, autoimmunity and

68 Thus, the onset of overt immune deficiency appears to be intimately linked with C

69 T cell homeostasis and it has been linked to immune deficiencies as well as autoimmune disorders.

70 n and bone marrow transplants, and inherited immune deficiencies as well as those on immunosuppressiv

71 HEJ factor, underlie certain severe combined immune deficiencies associated with defective V(D)J reco

72 on promote pathological conditions including immune deficiency, autoimmunity, and cancer.

73 of severe atopy, increased serum IgE levels, immune deficiency, autoimmunity, and motor and neurocogn

74 tations underlie a disorder of severe atopy, immune deficiency, autoimmunity, intellectual disability

75 IL-1R1(-/-) mice did not reflect a systemic immune deficiency, because immunized IL-1R1(-/-) mice su

76 astatic lung cancer model in Severe Combined Immune Deficiency-beige (SCID-bg) mice, should be attrib

77 onstitution of patients with severe combined immune deficiency, but the occurrence of leukaemia in a

78 for the most common forms of severe combined immune deficiency can lead to immune reconstitution in m

79 Thus, an innate immune deficiency can result in unstable gut microbiota

80 ic disorder causing cerebellar degeneration, immune deficiency, cancer predisposition, chromosomal in

81 weeks) or who had chronic pulmonary disease, immune deficiency, cardiac disease, or previous episodes

82 hronic granulomatous disease is an inherited immune deficiency caused by the absence of one of the co

83 ction with a recent biomarker of more severe immune deficiency (CD4 count <200 cells/mL) had a 44% in

84 efects in vitro, which is consistent with an immune deficiency cellular phenotype.

85 nic granulomatous disease (CGD) is a primary immune deficiency characterized by a defect in reactive

86 We conclude that mutations in LRBA cause an immune deficiency characterized by defects in B cell act

87 granulomatous disease (CGD) is an inherited immune deficiency characterized by increased susceptibil

88 riable immune deficiency (CVID) is a primary immune deficiency characterized by low levels of serum i

89 Common variable immunodeficiency is a rare immune deficiency, characterized by low levels of serum

90 del were older age, male sex, comorbidities (immune deficiency, cirrhosis), Knaus C/D score, and high

91 c regeneration and may be useful in treating immune deficiency conditions.

92 and convey to patients with common variable immune deficiency (CVID) an increased risk for autoimmun

93 ated with IgA deficiency and common variable immune deficiency (CVID) in humans.

94 Common variable immune deficiency (CVID) is a primary immune deficiency

95 Common variable immune deficiency (CVID) is an assorted group of primary

96 , and are best classified as common variable immune deficiency (CVID), although other genes, includin

97 ated in 10% of patients with common variable immune deficiency (CVID).

98 hip between the underlying diagnoses, severe immune deficiency, cytoreductive regimen, and graft-vers

99 The most severe primary immune deficiency diseases (PIDs) have been successfully

100 an centers studying therapy for rare primary immune deficiency diseases (PIDs), including severe comb

101 Primary immune deficiency diseases arise due to heritable defect

102 For many years, severe combined immune deficiency diseases, which are characterized by v

103 inked hyper IgM syndrome (XHM) is a combined immune deficiency disorder caused by genetic alterations

104 th X-linked hyper-IgM syndrome, an inherited immune deficiency disorder caused by mutations in the ge

105 rich syndrome (WAS) is an X-linked recessive immune deficiency disorder characterized by thrombocytop

106 Persons with primary immune deficiency disorders (PIDD) who receive oral poli

107 Complication rates are increased by immune deficiency disorders, malnutrition, vitamin A def

108 iciency virus (HIV) infection or other known immune deficiency disorders.

109 plantation (HSCT) is followed by a period of immune deficiency due to a paucity in T-cell reconstitut

110 now report on 5 unrelated cases of combined immune deficiency due to hypomorphic RAG mutations, and

111 An acquired immune deficiency due to interferon gamma (IFN-gamma) au

112 T cell memory and offers a new mechanism for immune deficiency during persistent viral infections.

113 s are discovered that cause various forms of immune deficiency, each with their consequent type of in

114 Ectodermal dysplasia with immune deficiency (EDI) is an immunological and developm

115 Ectodermal dysplasia with immune deficiency (EDI) is caused by alterations in NEMO

116 ning the primary features of common variable immune deficiency, exquisite vulnerability to infection

117 stics from 473 subjects with common variable immune deficiency followed over 4 decades in New York we

118 Severe immune deficiency follows autologous stem cell transplan

119 ompted the Medical Advisory Committee of the Immune Deficiency Foundation to issue recommendations ba

120 itution in nonobese diabetic severe combined immune deficiency gamma-/- mice reconstituted with human

121 d either by a distinct pathway involving the immune deficiency gene (imd) or by combined activation o

122 s formation in patients with severe combined immune deficiency, Glanzmann's thrombasthenia, Hermansky

123 and can be characterized by neuropathology, immune deficiency, growth retardation or predisposition

124 including Evans syndrome and common variable immune deficiency, have been found to have ALPS.

125 Along with this immune deficiency, HIV-1 has been linked to a number of

126 n of individuals with atopic skin disease or immune deficiency, however, can lead to persistent viral

127 iptericin reporters in the fat body required immune deficiency (imd) and domino.

128 identified homologs to all of the Drosophila immune deficiency (IMD) and Toll pathway components, an

129 on the activation of two signaling pathways: immune deficiency (IMD) and Toll.

130 iption factor Relish, a key regulator of the Immune deficiency (Imd) IIR signaling pathway.

131 ssociated with deregulated activation of the immune deficiency (IMD) pathway of host defense and can

132 The insect immune deficiency (IMD) pathway resembles the tumour nec

133 These responses depend on the immune deficiency (IMD) pathway via activation of the NF

134 The Drosophila immune deficiency (IMD) pathway, homologous to the mamma

135 The Drosophila immune deficiency (IMD) pathway, homologous to the mamma

136 t this bacterial strain, which activates the immune deficiency (IMD) pathway, is able to replicate an

137 lutionarily conserved signaling cascade, the immune deficiency (Imd) pathway, which activates NF-kapp

138 h as peptidoglycans, stimulates the Toll and immune deficiency (Imd) pathways to induce antimicrobial

139 and fungal pathogens is mediated by Toll and immune deficiency (Imd) pathways, but little is known ab

140 factors of the NF-B family, the Toll and the immune deficiency (IMD) pathways.

141 the innate immune response by activating the immune deficiency (imd) signaling cascade, a NF-kappaB-d

142 c inflammation that leads to deregulation of immune deficiency (IMD) signaling in the midgut of old a

143 of pgrp-lb results in the activation of the immune deficiency (IMD) signaling pathway and leads to t

144 In Drosophila, immune deficiency (IMD) signalling (tumour necrosis fact

145 flora and is inhibited by the activities of immune deficiency (Imd), JAK/STAT, and Leu-rich repeat i

146 on innate immune responses controlled by the immune deficiency (IMD), Toll, and other immune signalin

147 Although immune-deficiency (IMD)-mediated immune responses were o

148 fection is causal or secondary to underlying immune deficiencies in CD patients.

149 a cellular dipeptidase implicated in primary immune deficiencies in humans.

150 nd offers a biochemical explanation for rare immune deficiencies in man.

151 alcohol exposure also results in significant immune deficiencies in naive neonates.

152 Together these results show that immune deficiencies in this congenital neutropenia syndr

153 , may have potential use in the treatment of immune deficiency in alcoholic and nonalcoholic patients

154 of T lymphocytes, suggesting a basis for the immune deficiency in Bloom's syndrome.

155 nutrition (PEM), a common cause of secondary immune deficiency in children, is associated with an inc

156 If immune activation drives progressive immune deficiency in chronic HIV-1 infection, these acti

157 The mechanisms underlying immune deficiency in diabetes are largely unknown.

158 ystem, and possibly, to overcome age-related immune deficiency in general.

159 oid cell function shows no evidence of overt immune deficiency in HED mutant mice.

160 kine receptor deficiencies may contribute to immune deficiency in HIV-infected patients, and gamma -c

161 CVID), the most frequent symptomatic primary immune deficiency in humans, is a heterogeneous group of

162 onstrated multiple phenotypic expressions of immune deficiency in patients with nearly identical geno

163 on between the expansion of T(reg) cells and immune deficiency in the old, and that depletion of thes

164 animals are growth-retarded and show various immune deficiencies including a specific reduction in th

165 meostasis using a zebrafish model of primary immune deficiency induced by the human inhibitory Rac2D5

166 assess whether they might contribute to the immune deficiency induced by their T-cell-tropic parenta

167 trol of CMV infection, and correction of the immune deficiency induced by transplant is now clinicall

168 There was little evidence of immune deficiency, infection, or toxicity.

169 icin as well as other genes regulated by the immune deficiency innate immunity signaling pathway.

170 pressor 1), a negative regulator of the Imd (immune deficiency) innate immune-response pathway.

171 t features include congenital heart disease, immune deficiency, intermittent hypoglycemia, cognitive

172 fingers and toes, congenital heart disease, immune deficiency, intermittent hypoglycemia, cognitive

173 Systemic and local immune deficiency is associated with cancer, and the rol

174 Such severe immune deficiency is caused by multiple and profound B c

175 Because a host afflicted with an immune deficiency is not likely to purge a persistent in

176 3 gene encoding kindlin-3 underlie the human immune deficiency known as leukocyte adhesion deficiency

177 ive multicenter cohort: the French Childhood Immune Deficiency Long-term Cohort.

178 Alternatively, underlying immune deficiencies may emerge.

179 aise the possibility that cell type-specific immune deficiency may alter latency in distinct and impo

180 Subjects with common variable immune deficiency may have mutations in transmembrane ac

181 lammation supports the notion that an innate immune deficiency might underlie some instances of infla

182 s in mice homozygous for the severe combined immune deficiency mutation in the protein kinase, DNA-ac

183 iseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared wi

184 vessels of nonobese diabetic/severe combined immune deficiency (NOD/SCID) mice.

185 The immune deficiency of human immunodeficiency virus (HIV)

186 For patients with immune deficiency or leukemia WBC should be persistently

187 that are not accompanied by severe systemic immune deficiency or skewing.

188 o identifying a model of p22(phox)-dependent immune deficiency, our study indicates that a clinically

189 or required for activation of the Drosophila immune deficiency pathway by monomeric Gram-negative pep

190 plex prevents constitutive activation of the immune deficiency pathway in response to commensal micro

191 glycan and prevent the induction of tsetse's Immune Deficiency pathway that otherwise can damage the

192 ins (PGRPs) LCa and LCx, which activates the immune deficiency pathway.

193 oll and peptidoglycan recognition protein LC/immune deficiency (PGRP-LC/IMD) signaling pathways.

194 rize the recent advances in treating primary immune deficiency (PID) disorders by stem cell transplan

195 th the burgeoning field of genetics, primary immune deficiencies (PIDs) can be diagnosed and treated

196 Recent studies in patients with primary immune deficiencies (PIDs) have led to important breakth

197 nse variants in a gene implicated in primary immune deficiency, PLCG2, and a negative regulator of in

198 mphoproliferative disease, but the extent of immune deficiency related to R in patients who received

199 , possibly explaining, at least in part, the immune deficiency reported in some FA patients.

200 will help understand the relevance of innate immune deficiencies responsible for the higher risk of f

201 Innate immune deficiencies result in a spectrum of severe clini

202 ications for the management of patients with immune deficiencies resulting from malnutrition and irra

203 immune deficiencies such as severe combined immune deficiency (SCID) and X-linked agammaglobulinemia

204 ne deaminase (ADA)-deficient severe combined immune deficiency (SCID) may be treated by allogeneic he

205 34(+) cells in the hearts of severe combined immune deficiency (SCID) mice after experimental MI and

206 positive cells injected into severe combined immune deficiency (SCID) mice can transform into cardiom

207 ed by HIV-1 clades using the severe combined immune deficiency (SCID) mouse HIV encephalitis model, w

208 p to one-third of the normal severe combined immune deficiency (SCID) mouse life span, although they

209 ll line MCF-7 were used in a severe combined immune deficiency (SCID) mouse xenograft model to charac

210 designed against an X-linked severe combined immune deficiency (SCID) mutation in the IL2Rgamma gene

211 with one form of hereditary severe combined immune deficiency (SCID) syndrome are defective in store

212 ting in a form of hereditary severe combined immune deficiency (SCID) syndrome.

213 ns that range from T(-) B(-) severe combined immune deficiency (SCID) to Omenn syndrome.

214 inical phenotypes, including severe combined immune deficiency (SCID), autoimmunity, and inflammation

215 y diseases (PIDs), including severe combined immune deficiency (SCID), Wiskott-Aldrich syndrome (WAS)

216 the clinical presentation of severe combined immune deficiency (SCID).

217 T) for infants with X-linked severe combined immune deficiency (SCID-X1) lacking a matched sibling do

218 ors that is used to prevent or treat primary immune deficiency, several infectious diseases, and auto

219 Eventually, testing for immune deficiencies should more routinely follow a confi

220 omologue for caspar, a negative regulator of immune deficiency signaling pathway.

221 te whether a secondary functional neurogenic immune deficiency (spinal cord injury-induced immune def

222 e diseases, malignant diseases, and acquired immune deficiency states (e.g., after organ transplantat

223 therapeutic strategies for the treatment of immune deficiency states, and modulation of exaggerated

224 a range of new, genetically defined, primary immune deficiency states; and (iii) experimental infecti

225 erative BM depression/failure during complex immune deficiencies such as AIDS.

226 es of autoimmune etiology often present with immune deficiencies such as lymphopenia.

227 loss of gene function, resulting in primary immune deficiencies such as severe combined immune defic

228 ute respiratory syndrome (SARS) and acquired immune deficiency syndrome (AIDS) are just two of the mo

229 The acquired immune deficiency syndrome (AIDS) epidemic was first rec

230 our perception of HIV infection and acquired immune deficiency syndrome (AIDS) has changed from an al

231 Diarrhea in patients with acquired immune deficiency syndrome (AIDS) has proven to be both

232 or high-titer viral replication and acquired immune deficiency syndrome (AIDS) progression.

233 SCD criteria, 131 (57%) were due to acquired immune deficiency syndrome (AIDS), 25 (11%) were due to

234 ection on mortality from HIV and/or acquired immune deficiency syndrome (AIDS), and hepatitis or live

235 y in immunocompromised persons with Acquired Immune Deficiency Syndrome (AIDS), and to the developmen

236 ion of human immunodeficiency virus/acquired immune deficiency syndrome (AIDS), but the mechanism(s)

237 human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS), effectively serving a

238 human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS), hepatitis C, bovine s

239 portions, timing, and predictors of acquired immune deficiency syndrome (AIDS)-related and non-AIDS-r

240 an immunodeficiency virus (HIV) and acquired immune deficiency syndrome (AIDS).

241 an Immunodeficiency Virus (HIV) and Acquired Immune Deficiency Syndrome (AIDS).

242 l nervous system (CNS) pathology of acquired immune deficiency syndrome (AIDS).

243 isease epidemics, including that of acquired immune deficiency syndrome (AIDS).

244 that drives clinical progression to acquired immune deficiency syndrome (AIDS).

245 lcoholism along with other factors (acquired immune deficiency syndrome [AIDS]-defining events, hepat

246 ges from 17 macaques that developed acquired immune deficiency syndrome after infection with SIVsmmFG

247 d in the plasma of individuals with acquired immune deficiency syndrome and Kaposi sarcoma.

248 samples from an infant with severe combined immune deficiency syndrome and persistent RSV infection.

249 human immunodeficiency virus (HIV)/acquired immune deficiency syndrome are complex and not completel

250 the progressive immunodeficiency of acquired immune deficiency syndrome are controversial, particular

251 negative controls and patients with acquired immune deficiency syndrome but without encephalitis, as

252 eurodegenerative diseases including acquired immune deficiency syndrome dementia complex.

253 itive individual who presented with acquired immune deficiency syndrome despite repeatedly negative H

254 In a single-center study, all HIV/acquired immune deficiency syndrome patients with complete clinic

255 rus encephalitis causes dementia in acquired immune deficiency syndrome patients.

256 nfection in 25% of immunosuppressed acquired immune deficiency syndrome patients.

257 s, and human immunodeficiency virus/acquired immune deficiency syndrome therapy, mechanical hyperalge

258 d, potentially, as components of an acquired immune deficiency syndrome vaccine.

259 ious 12 months and who did not have acquired immune deficiency syndrome were sequentially enrolled fr

260 deficiency virus (HIV) causes AIDS (acquired immune deficiency syndrome), a disease in which the immu

261 cacy in the rhesus macaque model of acquired immune deficiency syndrome, enabling 50% of vaccinated m

262 mmune deficiency (spinal cord injury-induced immune deficiency syndrome, SCI-IDS) may account for the

263 ciency virus type 1 (HIV-1) develop acquired immune deficiency syndrome-associated dementia complex (

264 or epidemiologic studies of HIV and acquired immune deficiency syndrome.

265 ion of CD4(+) T cells that leads to acquired immune deficiency syndrome.

266 ranscriptase inhibitors (NRTIs) for acquired immune deficiency syndrome.

267 ss often from progression of HIV to acquired immune deficiency syndrome.

268 CD4 T cells, as takes place during acquired immune deficiency syndrome.

269 gy of various malignancies in human acquired immune deficiency syndrome.

270 in addition to immunodeficiency and acquired immune deficiency syndrome.

271 human immunodeficiency virus (HIV)/acquired immune deficiency syndrome; however, liver pathology dat

272 physiologic stressors and can contribute to immune deficiencies that occur in a variety of clinical

273 the ongoing gene therapy trials for primary immune deficiencies, the first reports of new trials and

274 The persistence of immune deficiency throughout life suggests that the cell

275 iency, ranging from T(-)B(-) severe combined immune deficiency to delayed-onset disease with granulom

276 une dysregulation, such as combined variable immune deficiency, transporter associated with antigen p

277 ed in patients with X-linked Severe Combined Immune Deficiency treated with gene therapy because of r

278 The Primary Immune Deficiency Treatment Consortium (PIDTC) is a coll

279 The Primary Immune Deficiency Treatment Consortium (PIDTC) is a netw

280 The Primary Immune Deficiency Treatment Consortium (PIDTC) is enroll

281 The Primary Immune Deficiency Treatment Consortium attempted to deve

282 The Primary Immune Deficiency Treatment Consortium was formed to ana

283 an Society for Immunodeficiency, and Primary Immune Deficiency Treatment Consortium.

284 That an engineered innate immune deficiency ultimately results in spontaneous inte

285 -type plasminogen activator, severe combined immune deficiency (uPA-SCID) mice" (chimeric mice).

286 duals suggesting its potential role in human immune deficiency virus (HIV)-associated neuroinflammati

287 The human immune deficiency virus type 1 (HIV-1) matrix protein p1

288 des, which could detect anti-p17 (HIV, human immune deficiency virus) antibodies (Ab) in phosphate bu

289 Human Immune Deficiency Virus-1 (HIV-1) infection can induce s

290 Immune deficiency viruses such as SIV in macaques or HIV

291 w of the literature regarding PML in primary immune deficiencies we found 26 cases, only 54% of which

292 Preexisting conditions related to immune deficiency were reported for 16% of deaths in the

293 ral PIDs, including forms of severe combined immune deficiency, Wiskott-Aldrich syndrome, and chronic

294 rted as a monogenic cause of common variable immune deficiency with features of immune dysregulation.

295 E syndrome, Job syndrome, HIES) is a complex immune deficiency with multiorgan clinical manifestation

296 umans, manifesting by a progressive combined immune deficiency with severe autoimmune disease.

297 y EXTL3 mutations as a novel cause of severe immune deficiency with skeletal dysplasia and developmen

298 three patients with X-linked severe combined immune deficiency (X-SCID) in gene-therapy trials using

299 aretroviral gene therapy for severe combined immune deficiency-X1, adenosine deaminase-deficient form

300 that disrupt TCR signaling can cause severe immune deficiency, yet less disruptive variants are some