ライフサイエンスコーパス: AAT

1 AAT and myeloperoxidase seemed to interact strongly with

2 AAT blocked the nuclear translocation of NF-kappaB p50/p

3 AAT decreased IkappaBalpha polyubiquitination linked thr

4 AAT deficiency (AATD) is associated with early-onset emp

5 AAT did not inhibit the proteasome; however, it altered

6 AAT inclusions were detected in liver biopsies of 63% of

7 AAT is mainly produced in the liver and functions to pro

8 AAT suppressed blood-mediated coagulation pathways by di

9 AAT treatment reduced the expansion of alloreactive T ef

10 AAT-AMS allowed AAL de-labeling in 98 (83%) patients-56%

11 AAT-AMS was implemented at two large Australian hospital

12 AAT-based therapy has the potential to improve graft sur

13 AAT-treated cells displayed enhanced chemokine-dependent

14 AAT-treated mice showed reduced serum tumor necrosis fac

15 AAT-treated stimulated DC displayed reduced MHC class II

16 AATs highly expressed in roots are good candidates for a

17 AATs in TC (TaAAP2 and TaAAP19) and SE (TaAAP13) may pla

18 ts suggest that i.m. delivery of rAAV type 1-AAT (rAAV1-AAT) induces a T regulatory response that all

19 ll patients with COVID-19, increases in IL-6:AAT predicted prolonged ICU stay and mortality, whereas

20 overwhelmed in severe illness, with the IL-6:AAT ratio markedly higher in patients requiring ICU admi

21 y and mortality, whereas improvement in IL-6:AAT was associated with clinical resolution (P < 0.0001)

22 pression from resistant codon-optimized AAT (AAT-opt) transgene cassette using adeno-associated virus

23 AAT levels were tested, including using AAV AAT-opt transgene cassettes targeted to muscle and liver

24 re consistent with the concept that abnormal AAT protein conformation and intrahepatic accumulation h

25 evels of AAT and both soluble and aggregated AAT protein in the liver.

26 In aspartate aminotransferase (AAT), an extended hydrogen bond network is coupled to th

27 We aimed to evaluate the impact of an AAT-AMS program on AAL prevalence, antibiotic usage, and

28 risk of low retinol [0.75 (0.62, 0.89)], and AAT with a lower risk of low plasma zinc [0.83 (0.70, 0.

29 of EFV monotherapy, 103N mutations (AAC and AAT) rapidly emerged and increased in the population to

30 splantation course of patients with AATD and AAT-replete COPD.

31 of overall rates of FEV1 decline in AATD and AAT-replete patients with COPD showed no significant dif

32 e interactions among DC, CD4(+) T cells, and AAT in vitro and in vivo.

33 d islet function was analyzed in control and AAT treated hosts.

34 in resolving exudates identified HX, GSN and AAT as potential leads for new drug discovery programs.

35 balance between lipoproteins, proteases, and AAT in atherosclerosis.

36 combination vectors carrying piZZ shRNA and AAT-opt transgenes separately, or a single bicistronic A

37 alpha-1 Antitrypsin (AAT) deficiency (AATD) is characterized by destruction o

38 Alpha-1 antitrypsin (AAT) deficiency (AATD) is characterized by neutrophil-dr

39 In this study we used alpha-1 antitrypsin (AAT) deficiency with the piZZ mutant phenotype as a mode

40 ines from patients with alpha-1 antitrypsin (AAT) deficiency, for which there is currently no drug or

41 s from mutations in the alpha-1 antitrypsin (AAT) gene.

42 alpha-1 antitrypsin (AAT) has been shown to reduce inflammatory markers, prom

43 Alpha-1 antitrypsin (AAT) is an acute phase protein that possesses immune-reg

44 s, we demonstrated that alpha-1 antitrypsin (AAT; Prolastin-C), a serine protease inhibitor used for

45 alpha(1)-Antitrypsin (AAT) deficiency is an underrecognized genetic condition

46 another serpinopathy, alpha(1)-antitrypsin (AAT) deficiency.

47 alpha(1)-Antitrypsin (AAT) encoded by the SERPINA1 gene is an acute-phase prot

48 ), neopterin (NEO), and alpha-1-antitrypsin (AAT) concentrations from asymptomatic fecal samples aver

49 Because alpha-1-antitrypsin (AAT) decreases HIV replication in PBMCs and monocytic ce

50 Alpha-1-antitrypsin (AAT) deficiency (AATD) is a genetic disease, caused by m

51 Alpha 1-antitrypsin (AAT) deficiency arises from an inherited mutation in the

52 the monogenic disorder alpha-1-antitrypsin (AAT) deficiency.

53 fects of treatment with alpha 1-antitrypsin (AAT) in a syngeneic nonautoimmune islet graft model.

54 Alpha-1-antitrypsin (AAT) is synthesized and secreted mainly by hepatocytes,

55 relation between plasma alpha-1-antitrypsin (AAT) levels in human donors and the development of acute

56 ease relevant inhibitor alpha-1-antitrypsin (AAT) Z-variant with catalytically inactive elastase.

57 tions of the biomarkers alpha-1-antitrypsin (AAT), myeloperoxidase, and neopterin.

58 alpha1 -Antitrypsin (AAT) deficiency is one of the most common genetic disord

59 The rationale of alpha1-antitrypsin (AAT) augmentation therapy to treat progressive emphysema

60 Mutations in alpha1-antitrypsin (AAT) can cause the protein to polymerise and be retained

61 our cell-based models of alpha1-antitrypsin (AAT) deficiency, unlike the case for FENIB, we were unab

62 alpha1-Antitrypsin (AAT) is a potent protease inhibitor, deficiency of which

63 alpha1-antitrypsin (AAT) regulates the activity of multiple proteases in the

64 ibution of two misfolded alpha1-antitrypsin (AAT) variants responsible for AAT deficiency disease: nu

65 the proteomic analysis, alpha1-antitrypsin (AAT), hemopexin (HX), and gelsolin (GSN), and tested aga

66 ylated variants of human alpha1-antitrypsin (AAT), Null Hong Kong (NHK), and Z (ATZ), in Man1b1 knock

67 r 1 (SERPINA1) encoding alpha-1 antitrypsin [AAT; p.V213A; P = 5.99E-9, odds ratio (OR) = 1.22] and c

68 erine protease inhibitor alpha-1 antitrysin (AAT).(2)

69 s that the mildly polymerogenic I (Arg39Cys) AAT mutant forms aberrant inter- and intra-molecular dis

70 Because AAT is naturally occurring and available clinically, exa

71 Because AAT therapy exerts antiinflammatory and immune modulator

72 individuals suggesting that affinity between AAT and elastase is strongly modulated by so-far overloo

73 No association was found between AAT concentration and outcomes for SZ-AATD.

74 results indicate that the interplay between AAT, NE, and lipoprotein particles is modulated by the g

75 ly prevent liver pathology and restore blood AAT concentration in AAT deficiencies.

76 To restore blood AAT levels in AAV8/shRNA-treated mice, several strategie

77 Q0(bolton)-AAT bound IL-8 and leukotriene B(4), comparable to healt

78 f HEK-293 significantly increased Q0(bolton)-AAT mRNA expression (p = 0.03) and Q0(bolton)-AAT trunca

79 confirmed the ability of purified Q0(bolton)-AAT protein to bind neutrophil elastase and to inhibit p

80 that augment levels of functional Q0(bolton)-AAT protein, thus offering a potential therapeutic optio

81 AT mRNA expression (p = 0.03) and Q0(bolton)-AAT truncated protein secretion (p = 0.04).

82 and anti-inflammatory capacity of Q0(bolton)-AAT.

83 e been employed for the gene therapy of both AAT-deficient lung disease and liver disease.

84 of healthy humans, were similarly reduced by AAT or rAAT; human neutrophils adhering to endothelial c

85 ilitate automated quantification of cellular AAT accumulation using a 96-well immunofluorescence read

86 Lack of functioning circulating AAT protein also causes uninhibited elastolytic activity

87 ility to efficiently develop tools to combat AAT.

88 pulation in muscle biopsy samples containing AAT-expressing myofibers.

89 he Null Bolton allele (Q0(bolton) ) contains AAT protein of truncated size.

90 In contrast, AAT suppressed LPS-induced in vitro secretion of proinfl

91 to 100-fold lower than native plasma-derived AAT.

92 ducible functional CCR7 is maintained during AAT-mediated anti-inflammatory conditions.

93 ower (80%, P < 0.001) when exposed to either AAT or rAAT.

94 decreased by 60-80% (P < 0.001) with either AAT or rAAT.

95 ded standard dose (SD; 60 mg/kg/wk) elevates AAT trough serum levels to around 50% of normal; however

96 In this review, we examine AAT resistance in GBMs, with an emphasis on six potentia

97 e lower levels of serum transaminases, fewer AAT inclusions in liver, and lower liver stiffness than

98 nt potential as novel therapeutic agents for AAT.

99 1-antitrypsin (AAT) variants responsible for AAT deficiency disease: null Hong Kong (NHK) and Z allel

100 er were highly statistically significant for AAT (28.71; P < 0.0001) and myeloperoxidase (62.79; P <

101 he hepatocyte, which is the primary site for AAT production.

102 ocyte transplantation may be therapeutic for AAT-Z liver disease and may provide an alternative to pr

103 n therapy"-represents a specific therapy for AAT deficiency and raises serum levels above the protect

104 ic mRNA therapy as a potential treatment for AAT deficiency.

105 ealed that the selective endocytosis of four AATs during starvation required the alpha-arrestin famil

106 for individuals with pulmonary disease from AAT deficiency (AATD).

107 hypoxia plays a key role in GBM escape from AAT.

108 cytes in culture, including hepatocytes from AAT deficient patients.

109 udied, 2 that related to intestinal function-AAT and myeloperoxidase-were associated with small but h

110 ce, several strategies to restore functional AAT levels were tested, including using AAV AAT-opt tran

111 Furthermore, AAT-ASO administration in these animals stopped liver di

112 Here we show that clinical grade AAT (with elastase inhibitory activity) and a recombinan

113 sense oligonucleotide targeted against hAAT (AAT-ASO) and found reductions in circulating levels of A

114 However, AAT demonstrates transient effects because many patients

115 iZ transgenic mouse strain expresses a human AAT (hAAT) transgene that contains the AATD-associated G

116 nt were examined using OT-II cells and human AAT (0.5 mg/ml).

117 We tested modified mRNA encoding human AAT in primary human hepatocytes in culture, including h

118 ined and donor-derived cells expressed human AAT protein.

119 ll lines stably expressing a wild type human AAT (MAAT) and a disease-causing polymer-forming variant

120 r transplantation is augmentation with human AAT protein pooled from sera, which is only reserved for

121 s, treatment of transplant donors with human AAT resulted in an increase in interleukin-10 messenger

122 tributes to the intracellular retention of I AAT.

123 ed by the gate region around position 213 in AAT, far away from the unaltered reactive center loop (3

124 t strategies, may therefore be beneficial in AAT deficiency-associated liver disease.

125 es directly enhance the rate of catalysis in AAT.

126 ology and restore blood AAT concentration in AAT deficiencies.

127 Ablation of DCs (in AAT-treated CD11c-DTR donors) decreased CD4(+)CD25(+)Fox

128 e achieved high gene-targeting efficiency in AAT-deficiency patient iPSCs with 25%-33% of the clones

129 on therapy to treat progressive emphysema in AAT-deficient patients is based on inhibition of neutrop

130 rotein replacement for treating emphysema in AAT-ZZ individuals.

131 es with the enhancement in catalytic rate in AAT.

132 egulator of the unfolded protein response in AAT-deficient monocytes, and epigenetic silencing of its

133 ,184 differentially expressed transcripts in AAT-treated hosts at 3 d posttransplantation.

134 ecognize C-terminal acidic sorting motifs in AATs and thereby instructs Rsp5 to ubiquitinate proximal

135 Z mice exhibit many AATD symptoms, including AAT protein aggregates, increased hepatocyte death, and

136 c levels of AAT, whereas levels of insoluble AAT varied among individuals.

137 An integrated AAT-AMS program was effective in both de-labeling of AAL

138 further doubts over the need for intravenous AAT augmentation in this cohort.

139 onale: Augmentation therapy with intravenous AAT (alpha-1 antitrypsin) is the only specific therapy f

140 pon exposure of CD4(+) T cells to OVA-loaded AAT-treated DC, 2.7-fold more Foxp3(+) Treg cells were o

141 ung destruction and early emphysema in a low AAT, and high neutrophil elastase environment in the lun

142 and development of emphysema, caused by low AAT levels and a high neutrophil burden in the airways o

143 r is abnormal, but not in those expressing M AAT.

144 essing normal, M-type alpha-1 antitrypsin (M-AAT) to AAT-deficient subjects at various doses by multi

145 triene B(4), comparable to healthy control M-AAT, and significantly decreased leukotriene B(4)-induce

146 Thus, via altered metabolism, AAT exerts effective GVHD protection while enhancing GVL

147 We demonstrate that in AATD, misfolded AAT protein accumulates in the endoplasmic reticulum of

148 Also, N-glycans attached to misfolded AAT are not required for accelerated degradation mediate

149 utcomes when initiated after starting modern AAT.

150 The Z allele of SERPINA1 encodes a mutant AAT, named ATZ, that changes the protein structure and l

151 mapped to a peptide in the endogenous mutant AAT protein that contained a common polymorphism not inc

152 es, we observed over 90% knockdown of mutant AAT with a 13- to 30-fold increase of circulating wild-t

153 ted iPSCs were functional without the mutant AAT accumulation.

154 The mutant AAT protein aggregates and accumulates in the liver lead

155 Accumulation of mutated AAT protein aggregates in hepatocytes leads to endoplasm

156 se-deficient mice treated with either native AAT or rAAT exhibited significant reductions in infiltra

157 MAAT and cell lines expressing ZAAT and NHK AAT mutants.

158 ariant (ZAAT) and the truncated variant (NHK AAT), we measured basal intracellular free Ca(2+), its r

159 0% reduction in levels of circulating normal AAT, demonstrating potential for this approach in higher

160 that can be further improved by normalizing AAT levels.

161 aluate the biological effects of normalizing AAT trough levels with double-dose (DD) therapy (120 mg/

162 ctions enhance intracellular accumulation of AAT mutants and implicate the oxidative ER state as a pa

163 er, the mechanism behind these activities of AAT is poorly understood.

164 hind the favorable tolerogenic activities of AAT.

165 ence of FDC supernatant, but the addition of AAT at concentrations >0.5 mg/ml inhibited virus replica

166 data suggest that systemic administration of AAT can be a promising therapy to treat acute liver fail

167 Administration of AAT early after BMT decreased mortality in three models

168 e aimed to explore whether administration of AAT may represent a therapeutic strategy to treat acute

169 hese findings suggest that administration of AAT represents a novel unique and viable strategy to mit

170 Administration of AAT-ASO in nonhuman primates led to an approximately 80%

171 hepatocellular carcinoma, whereas decline of AAT levels in sera is responsible for pulmonary emphysem

172 The effect of AAT concentrations on outcomes was assessed in 82 SZ ind

173 The potent anti-inflammatory effect of AAT is possibly mediated by suppression of c-Jun N-termi

174 s, suggesting a cell type-specific effect of AAT.

175 odels of BMT, we have studied the effects of AAT on GvHD severity.

176 Therapeutic effects of AAT were evaluated by monitoring animal survival, histop

177 hibitory activity) and a recombinant form of AAT (rAAT) without anti-elastase activity reduces lung i

178 cking elastase inhibitory activity) forms of AAT were equally effective in preventing acute liver inj

179 ess, supporting the routine incorporation of AAT into AMS programs.

180 ated with 12 weekly intravenous infusions of AAT augmentation therapy before repeat imaging.

181 Intra-articular injection of AAT or GSN protected cartilage integrity in mice with in

182 ally 48 h after intra-articular injection of AAT or GSN.

183 nd found reductions in circulating levels of AAT and both soluble and aggregated AAT protein in the l

184 The expression levels of AAT homeologs showed unequal contributions in response t

185 ype did not have increased hepatic levels of AAT, whereas levels of insoluble AAT varied among indivi

186 a gene expression, were observed in lungs of AAT-deficient patients treated with AAT therapy compared

187 ce and the NSG-PiZ transgenic mouse model of AAT deficiency.

188 and the liver disease due to the Z mutant of AAT (ATZ) is a prototype of conformational disorder due

189 A mutant of AAT (E342K) called ATZ forms polymers that are present a

190 against the principal causative organisms of AAT: Trypanosoma congolense, and Trypanosoma vivax.

191 48-linked Ub-IkappaBalpha in the presence of AAT, correlating altered ubiquitination with a prolonged

192 In the presence of AAT, degradation of cytoplasmic IkappaBalpha was dramati

193 lammatory and immunomodulatory properties of AAT can be independent of elastase inhibition.

194 ted no increased risk of COPD, regardless of AAT concentration.

195 The acute phase response of AAT (alpha-1 antitrypsin) to COVID-19 was also evaluated

196 d novel mechanism and highlights the role of AAT augmentation therapy in ameliorating inflammation in

197 The production and sialylation of AAT increased in COVID-19, but this antiinflammatory res

198 pical corticosteroid use before the start of AAT (OR, 3.85; 95% CI, 1.35-11.03), a corneal ring infil

199 -29.67), and age >/=33 years at the start of AAT (OR, 4.02; 95% CI, 1.46-11.06).

200 other feature of AK) present at the start of AAT (OR, 5.89; 95% CI, 1.17-29.67), and age >/=33 years

201 findings were collected both at the start of AAT and subsequently at the time that topical corticoste

202 ee of scleritis and hypopyon at the start of AAT, topical corticosteroids were not associated with wo

203 ent of corticosteroid use after the start of AAT, was used to estimate the odds ratios (ORs) of a sub

204 costeroids had been used before the start of AAT.

205 ong patients with AATD is similar to that of AAT-replete patients with COPD, patients with AATD with

206 protease inhibitor used for the treatment of AAT deficiency, inhibits IBMIR and cytokine-induced infl

207 mice expressing human AATZ (the Z variant of AAT) confers any competitive advantages compared to host

208 AT expression from resistant codon-optimized AAT (AAT-opt) transgene cassette using adeno-associated

209 Exogenous administration of HX, GSN, or AAT abrogated the effects of IL-1beta and osteoarthritic

210 COPD at baseline, but not former smoking or AAT concentrations, predicted greater spirometry decline

211 ce that the putative protective threshold or AAT concentrations predict risk within the SZ genotype,

212 hat encodes the alpha-1 antitrypsin peptide (AAT), called the Pi*ZZ genotype, causes a liver and lung

213 pressing the common Z and other polymerising AAT variants where conformational behaviour is abnormal,

214 Post-AAT, prescribing of narrow-spectrum penicillins was more

215 spectrum of antibiotic courses pre- and post-AAT-AMS, and antibiotic appropriateness (using standard

216 AT-AMS) and 3 months following testing (post-AAT-AMS) were recorded for each participant.

217 ic usage for 12 months prior to testing (pre-AAT-AMS) and 3 months following testing (post-AAT-AMS) w

218 to antimicrobial stewardship (AMS) programs (AAT-AMS) is not widespread.

219 tion of a mutant alpha1-antitrypsin protein (AAT-Z) results in its accumulation within hepatocytes an

220 eatments for emphysema, infusion of purified AAT from pooled human plasma-so-called "augmentation the

221 d mainly by hepatocytes, and plasma purified AAT is used for augmentation therapy in patients with AA

222 that i.m. delivery of rAAV type 1-AAT (rAAV1-AAT) induces a T regulatory response that allows ongoing

223 ive clinical drugs were identified to reduce AAT accumulation in diverse patient iPSC-derived hepatoc

224 ld-type AAT protein from the shRNA-resistant AAT-opt cassette.

225 Systems-biology-based analysis revealed AAT down-regulated regulatory hubs formed by inflammatio

226 rminal acidic sorting motifs within the same AATs, which initiates exclusive substrate-induced endocy

227 Secreted AAT protein increased from 1,14 to 3,43 ug/ml in media f

228 A serum AAT level of 11 muM represents the protective threshold

229 cretion into the blood and causing low serum AAT levels ( approximately 3-7 muM with normal serum lev

230 isease and investigated the ability of serum AAT to control LTB4 signaling in neutrophils.

231 with advanced lung disease caused by severe AAT deficiency.

232 gnal differentiates between healthy and sick AAT-deficient individuals suggesting that affinity betwe

233 We found that endogenous SO2/AAT pathway existed in adipose tissues including perivas

234 M-specific AAT expression was observed in all subjects in a dose-de

235 Muscle biopsies at 1 year had sustained AAT expression and a reduction of inflammatory cells com

236 The ensuing systemic AAT deficiency leads to pulmonary emphysema, while intra

237 These data suggest that short-term AAT treatment of human islet transplant recipients may b

238 mes were dramatically improved by short-term AAT treatment.

239 incorporation of antibiotic allergy testing (AAT) into antimicrobial stewardship (AMS) programs (AAT-

240 demonstrate that AAT can bind LTB4 and that AAT/LTB4 complex formation modulates BLT1 engagement and

241 It is now apparent that AAT has important immune-regulatory roles that would be

242 We demonstrate that AAT can bind LTB4 and that AAT/LTB4 complex formation mo

243 In summary, this study demonstrates that AAT can regulate neutrophil apoptosis by a previously un

244 eases NF-kappaB activity, we postulated that AAT might also block FDC-mediated HIV replication.

245 l models, and it was recently suggested that AAT exerts antiapoptotic activities, we aimed to explore

246 typic and phenotypic differences between the AAT-relevant species of parasite or host and their model

247 racellular DC IL-10 was 2-fold higher in the AAT group.

248 n with engrafted hepatocytes occurred in the AAT-Z-expressing mice even in the absence of severe live

249 protein, resulting in the Z mutation of the AAT gene (ZAAT).

250 a genetic disease, caused by mutation of the AAT gene.

251 amage in the liver where the majority of the AAT protein is produced.

252 uble LT declined faster (P < 0.002) than the AAT-replete patients.

253 We learned that the AAT-specific T cells in this patient were cytolytic in p

254 Antiangiogenic therapy (AAT) is a treatment option that targets GBM-associated v

255 These AATs are also necessary to maintain intracellular ornith

256 increased, and (4) Aortic acceleration time (AAT)/AET, cardiac output and stroke volume were decrease

257 ormal, M-type alpha-1 antitrypsin (M-AAT) to AAT-deficient subjects at various doses by multiple i.m.

258 elastolytic activity in the lungs leading to AAT deficiency-related emphysema.

259 rapies for lung and liver diseases linked to AAT deficiency.

260 cular and biochemical basis of resistance to AAT in GBM patients.

261 Taken together, AAT significantly improves islet graft survival after in

262 s an ortholog of the human LAT1 transporter, AAT-1, imports Kyn into sites of KynA production.

263 tosis of sugar- and amino acid transporters (AATs) (Muller et al., 2015).

264 We describe these amino acid transporters (AATs).

265 nd Main Results: DD therapy increased trough AAT levels to normal and, compared with SD therapy, redu

266 A low-abundance, truncated AAT protein was confirmed in plasma of a Q0(bolton)-AATD

267 Animal African trypanosomiasis (AAT) is a significant socioeconomic burden for sub-Sahar

268 Animal African trypanosomiasis (AAT), caused by Trypanosoma congolense and Trypanosoma v

269 an serum-derived alphaalpha-1- anti-trypsin (AAT) reduces production of proinflammatory cytokines, in

270 ent increases in transgene-derived wild-type AAT after local intramuscular vector administration.

271 g., shRNA) and restore circulating wild-type AAT expression from resistant codon-optimized AAT (AAT-o

272 to 30-fold increase of circulating wild-type AAT protein from the shRNA-resistant AAT-opt cassette.

273 Z-type AAT molecules polymerize within the hepatocyte, precludi

274 Under AAT treatment (60 mg/kg), DLN contained twice more fluor

275 However, despite altering the ratio in vivo, AAT had no direct effects on either the donor T effector

276 Here, 283 full length wheat AAT genes representing 100 distinct groups of homeologs

277 However, no systematic analysis of wheat AAT genes has been reported to date.

278 treatment is liver transplantation, whereas AAT replacement therapy is therapeutic for emphysema.

279 , these results provide a mechanism by which AAT augmentation therapy impacts on LTB4 signaling in vi

280 dates for amino acid uptake from soil whilst AATs highly expressed in senescing leaves and stems may

281 ter risk of low ferritin was associated with AAT [1.19 (1.03, 1.37)] and NEO [1.22 (1.04, 1.44)].

282 cells) was maintained or even enhanced with AAT treatment of the donor, mediated by an expanded popu

283 addition, treatment of AATD individuals with AAT augmentation therapy decreased neutrophil ADAM-17 ac

284 nally, treatment of ZZ-AATD individuals with AAT augmentation therapy decreased plasma LTB4 concentra

285 Livers of PiZ mice and human patients with AAT deficiency were both found to have a severe perturba

286 of hepatocellular carcinoma in patients with AAT deficiency.

287 ed for augmentation therapy in patients with AAT deficiency.

288 between patients with AATD and patients with AAT-replete COPD (P > 0.09).

289 d January 2008, a total of 231 patients with AAT-replete COPD and 45 with AATD underwent LT at Clevel

290 effect is more pronounced for ATZ than with AAT and is only partially dependent on the glycan-bindin

291 transplantation when they were treated with AAT compared with mice treated with saline.

292 Concomitantly, mice treated with AAT showed significantly lower serum levels of tumor nec

293 lungs of AAT-deficient patients treated with AAT therapy compared with untreated patients.

294 e reduced in both mouse strains treated with AAT; significantly lower levels of these genes, as well

295 itioning and postconditioning treatment with AAT resulted in attenuation or prevention of GVHD and su

296 Systemic treatment with AAT significantly decreased Jo2-induced liver cell apopt

297 th FDCs or their supernatant with or without AAT, and ensuing viral RNA and p24 production were monit

298 l shift perturbations for methyl groups in Z AAT (E342K).

299 nt of cells with reducing agents increases Z AAT secretion.

300 s thought to be owing to toxicity from the Z-AAT mutant protein that folds poorly and forms insoluble