ライフサイエンスコーパス: antitrypsin

1 ation by alpha1-proteinase inhibitor (alpha1-antitrypsin).

2 romising the inhibitory activity of Z alpha1-antitrypsin.

3 of anti-inflammatory signalling by M alpha1-antitrypsin.

4 n-dependent degradation of misfolded alpha-1 antitrypsin.

5 prions, vasopressin receptor 2, and alpha-1-antitrypsin.

6 the dislocation of misfolded luminal alpha-1 antitrypsin.

7 his could be inhibited by addition of alpha1-antitrypsin.

8 and proteasomal degradation of mutant alpha1-antitrypsin.

9 ns stable at approximately 3.5 A in alpha(1)-antitrypsin.

10 protein, null Hong Kong variant of alpha(1)-antitrypsin.

11 and in beta-strand 1C compared with alpha(1)-antitrypsin.

12 hile no such movement is evident in alpha(1)-antitrypsin.

13 the pathological polymers formed by alpha(1)-antitrypsin.

14 on when compared to the wild-type M alpha(1)-antitrypsin.

15 ir effects on the shutter region of alpha(1)-antitrypsin.

16 ined several degradation products of alpha-1 antitrypsin.

17 ema caused by mutations in the serpin alpha1-antitrypsin.

18 ited by alpha-ACT but not by related alpha-1-antitrypsin.

19 nificant amounts of human albumin and alpha1-antitrypsin.

20 of polarity, and reduced secretion of alpha1-antitrypsin.

21 sease associated with the Z allele of alpha1-antitrypsin.

22 HD severity markers, calprotectin and alpha1-antitrypsin.

23 s fragmentation in cells expressing Z-alpha1-antitrypsin.

24 required for ubiquitination of mutant alpha1-antitrypsin, a luminal ERAD substrate.

25 showed an impressive 80% increase of alpha-1-antitrypsin, a target of miR-126-3p.

26 Alpha-1-antitrypsin (a1AT) deficiency is caused by homozygosity

27 on transplantation, human albumin and alpha1-antitrypsin (A1AT) in mouse sera secreted by encapsulate

28 ssion of the human protease inhibitor alpha1-antitrypsin (A1AT) in Nicotiana benthamiana.

29 atients heterozygous for an abnormal alpha-1 antitrypsin (A1AT) mutation may have an increased risk o

30 f this correlation and the effect of alpha-1-antitrypsin (A1AT) on the expression of the iron hormone

31 mutations in SERPINA1 coding for the alpha-1 antitrypsin (A1AT) protein is the only well established

32 alpha1-Antitrypsin (A1AT) purified from human plasma upregulate

33 This study shows that human alpha1-antitrypsin (A1AT) upregulates expression and release of

34 alpha1-Antitrypsin (A1AT) was identified as a plasma protease i

35 I), apolipoprotein C-III (ApoC-III), alpha-1-antitrypsin (A1AT), and alpha-2-HS-glycoprotein (A2HSG);

36 ove function with supplementation of alpha-1 antitrypsin (A1AT).

37 ined by proper circulating levels of alpha-1 antitrypsin (A1AT).

38 ers of the serine protease inhibitor alpha-1-antitrypsin (A1AT).

39 a point mutation (Glu342Lys) in the alpha(1)-antitrypsin (A1AT, also known as SERPINA1) gene that is

40 Human alpha-1-antitrypsin (A1PI) is a plasma protein with the function

41 The rationale of alpha1-antitrypsin (AAT) augmentation therapy to treat progress

42 Mutations in alpha1-antitrypsin (AAT) can cause the protein to polymerise an

43 roxidase (MPO), neopterin (NEO), and alpha-1-antitrypsin (AAT) concentrations from asymptomatic fecal

44 Because alpha-1-antitrypsin (AAT) decreases HIV replication in PBMCs and

45 Alpha-1-antitrypsin (AAT) deficiency (AATD) is a genetic disease

46 alpha-1 Antitrypsin (AAT) deficiency (AATD) is characterized by

47 Alpha-1 antitrypsin (AAT) deficiency (AATD) is characterized by

48 Alpha 1-antitrypsin (AAT) deficiency arises from an inherited mu

49 Alpha-1 antitrypsin (AAT) deficiency is a common single-gene dis

50 alpha(1)-Antitrypsin (AAT) deficiency is an underrecognized genet

51 alpha1 -Antitrypsin (AAT) deficiency is one of the most common g

52 Alpha-1 antitrypsin (AAT) deficiency is well-suited as a target

53 In this study we used alpha-1 antitrypsin (AAT) deficiency with the piZZ mutant phenot

54 Using iPSC lines from patients with alpha-1 antitrypsin (AAT) deficiency, for which there is current

55 Thus, in our cell-based models of alpha1-antitrypsin (AAT) deficiency, unlike the case for FENIB,

56 sociated with another serpinopathy, alpha(1)-antitrypsin (AAT) deficiency.

57 treatment for the monogenic disorder alpha-1-antitrypsin (AAT) deficiency.

58 alpha(1)-Antitrypsin (AAT) encoded by the SERPINA1 gene is an acu

59 e that results from mutations in the alpha-1 antitrypsin (AAT) gene.

60 alpha-1 antitrypsin (AAT) has been shown to reduce inflammatory

61 tudied the effects of treatment with alpha 1-antitrypsin (AAT) in a syngeneic nonautoimmune islet gra

62 alpha1-Antitrypsin (AAT) is a potent protease inhibitor, defici

63 Alpha-1 antitrypsin (AAT) is an acute phase protein that possess

64 Alpha-1-antitrypsin (AAT) is synthesized and secreted mainly by

65 n inverse correlation between plasma alpha-1-antitrypsin (AAT) levels in human donors and the develop

66 ect of the serine protease inhibitor alpha-1 antitrypsin (AAT) on IL-32 levels and showed suppression

67 The serum protease inhibitor alpha1-antitrypsin (AAT) possesses antiinflammatory properties

68 on of the serine proteinase inhibitor alpha1-antitrypsin (AAT) prevents type 1 diabetes development i

69 alpha1-antitrypsin (AAT) regulates the activity of multiple pro

70 soluble distribution of two misfolded alpha1-antitrypsin (AAT) variants responsible for AAT deficienc

71 on of the disease relevant inhibitor alpha-1-antitrypsin (AAT) Z-variant with catalytically inactive

72 We demonstrate that treatment with alpha1-antitrypsin (AAT), an agent that dampens inflammation, d

73 selected from the proteomic analysis, alpha1-antitrypsin (AAT), hemopexin (HX), and gelsolin (GSN), a

74 for concentrations of the biomarkers alpha-1-antitrypsin (AAT), myeloperoxidase, and neopterin.

75 lded N-glycosylated variants of human alpha1-antitrypsin (AAT), Null Hong Kong (NHK), and Z (ATZ), in

76 d human islets, we demonstrated that alpha-1 antitrypsin (AAT; Prolastin-C), a serine protease inhibi

77 amily A member 1 (SERPINA1) encoding alpha-1 antitrypsin [AAT; p.V213A; P = 5.99E-9, odds ratio (OR)

78 Deficiency of alpha(1) -antitrypsin (alpha(1) AT) may be a determinant of suscep

79 Homozygous (PIZZ) alpha-1-antitrypsin (alpha(1)-AT) deficiency is associated with

80 Alpha-1 antitrypsin (alpha(1)-AT) is a member of the serpin clas

81 mechanism of peptide modulation of alpha(1)-antitrypsin (alpha(1)-AT) polymerization and depolymeriz

82 imed to evaluate fecal calprotectin, alpha-1-antitrypsin (alpha(1)-AT), and elastase at the time of f

83 lding of the canonical serpin human alpha(1)-antitrypsin (alpha(1)-AT).

84 nd emphysema caused by mutations in alpha(1)-antitrypsin (alpha(1)AT), and thrombosis caused by mutat

85 tional dynamics of the serpin human alpha(1)-antitrypsin (alpha(1)AT).

86 Alpha-1 antitrypsin (alpha1-AT) deficiency is the most common ge

87 n serine protease inhibitor (serpin) alpha-1 antitrypsin (alpha1-AT) protects tissues from proteases

88 of three HNF-4alpha sensitive genes, alpha1-antitrypsin (alpha1-AT), transthyretin (TTR), and apolip

89 ecific to liver proteins: albumin and alpha1-antitrypsin (alpha1-AT).

90 alpha1-Antitrypsin (alpha1AT) deficiency (alpha1ATD) is a conse

91 Although alpha1-antitrypsin (alpha1AT) does not naturally inhibit contac

92 Point mutants of alpha1 -antitrypsin (alpha1AT) form ordered polymers that are re

93 ol region (LCR) upstream of the human alpha1-antitrypsin (alpha1AT) gene that is required for gene ac

94 Lys) in the serine protease inhibitor alpha1-antitrypsin (alpha1AT), which is found in more than 4% o

95 ented in a configuration resistant to alpha1-antitrypsin (alpha1AT).

96 family: protein C inhibitor (PCI) and alpha1-antitrypsin (alpha1AT); however, both exhibit poor react

97 ion could be recovered by addition of alpha1-antitrypsin, an endogenous inhibitor of serine proteases

98 ere that monomers of plasma serpins alpha(1)-antitrypsin and antithrombin are stable on incubation wi

99 568223361) was associated with lower alpha-1 antitrypsin and functional small airway disease (P = 0.0

100 ded, with significant accumulation of alpha1-antitrypsin and GRP78.

101 l-studied human serum glycoproteins, alpha-1-antitrypsin and immunoglobulin G.

102 ations stabilise the native fold of alpha(1)-antitrypsin and increase secretion of monomeric protein

103 cretion and secretion of endogenous alpha(1)-antitrypsin and serum albumin from HepG2 cells.

104 erine protease inhibitors [Serpina1a (alpha1-antitrypsin) and Elafin] was dysregulated in Fbln5(-/-)

105 pathy (calprotectin, myeloperoxidase, alpha1-antitrypsin) and the prevalence of bacterial but not vir

106 obin increased, whereas transferrin, alpha-1-antitrypsin, and apolipoprotein A-1 decreased.

107 genes such as SERPINA1, which encodes alpha1 antitrypsin, and FOXP4, an inhibitor of mucus production

108 R2, Bid), optimal IL-13 inhibition of alpha1-antitrypsin, and IL-13-induction of and activation of ca

109 es, the solubility of mutant forms of alpha1-antitrypsin, and interactions with newly synthesized gly

110 de placed at the N-terminus of human alpha-1 antitrypsin, and is named EAT.

111 nic antigen, retinol binding protein, alpha1-antitrypsin, and squamous cell carcinoma antigen-were co

112 d levels of carcinoembryonic antigen, alpha1-antitrypsin, and squamous cell carcinoma antigen.

113 of surfactant proteins A, B, and C, alpha-1-antitrypsin, and the cystic fibrosis transmembrane condu

114 odepleted of albumin, IgG, IgA, haploglobin, antitrypsin, and transferrin.

115 paraoxonase/arylesterase], SERPINA1 [alpha-1-antitrypsin], and APOF [apolipoprotein F]) were signific

116 23), elevated fecal concentration of alpha-1 antitrypsin (aOR: 4.82; 95% CI: 1.01, 25.29), and anemia

117 Serum levels of nine biomarkers (alpha1 antitrypsin, apolipoprotein CIII, brain-derived neurotro

118 ular serpins such as antithrombin and alpha1-antitrypsin are the quintessential regulators of proteol

119 tifying cathepsin C, cathepsin Z, and alpha1-antitrypsin as additional potential cargoes for LMAN1, n

120 e found, using alpha-1-acid glycoprotein and antitrypsin as model systems for surface glycans, that t

121 levels of inflammatory biomarkers and alpha1-antitrypsin at baseline.

122 scopy to patient-derived samples of alpha(1)-antitrypsin at natural isotopic abundance to investigate

123 Alpha-1-antitrypsin (AT) deficiency is the most common genetic c

124 In the classical form of alpha1-antitrypsin (AT) deficiency, a point mutation in AT alte

125 molecular basis of liver disease in alpha-1-antitrypsin (AT) deficiency.

126 ithelial cells with purified plasma M alpha1-antitrypsin attenuates this inflammatory response, openi

127 m due to accumulation of the mutant Z alpha1-antitrypsin (ATZ) and is a key example of an disease mec

128 unoglobulin G, transferrin, fibrinogen and a-antitrypsin), both in buffer and when spiked into human

129 e most similar to human glycoprotein alpha-1-antitrypsin, but with a remarkable functional diversific

130 the intracellular polymerization of Z alpha1-antitrypsin by 60%.

131 d the intracellular accumulation of Z alpha1-antitrypsin by 70% in a cell model of disease.

132 ers, followed by IL-2 receptor alpha, alpha1-antitrypsin, C-reactive protein, YKL-40, cellular fibron

133 d by alpha(1)-proteinase inhibitor (alpha(1)-antitrypsin), C1 inhibitor, and most efficiently by anti

134 globulin, zinc alpha-2 glycoprotein, alpha-1 antitrypsin, complement factor B, haptoglobin, transthyr

135 leukin-6, interleukin-8, and elastase-alpha1-antitrypsin complexes compared with presurgery levels (p

136 leukin-8, interleukin-6, and elastase-alpha1-antitrypsin complexes were elevated compared with contro

137 nterleukin-6, interleukin-8, elastase-alpha1-antitrypsin complexes, thrombin-antithrombin complexes,

138 ep sequencing study of SERPINA1 with alpha-1 antitrypsin concentrations in a heavy smoker and chronic

139 Alpha-1-antitrypsin concentrations partially mediate the seasona

140 ure using deep gene resequencing and alpha-1 antitrypsin concentrations.Methods: DNA samples from 1,6

141 nt with the serine protease inhibitor alpha1-antitrypsin decreased serum levels of HS, leading to a r

142 Alpha1-antitrypsin defciency-related liver disease is therefore

143 bile acid synthetic disorder (BASD), alpha-1 antitrypsin deficiency (A1AT), chronic intrahepatic chol

144 Liver disease due to alpha-1-antitrypsin deficiency (A1ATD) is associated with hepati

145 Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disease

146 Alpha-1 antitrypsin deficiency (AATD) is among the most common g

147 alpha(1)-Antitrypsin deficiency (AATD) is an inherited disease ch

148 Rationale: The ZZ genotype of alpha-1 antitrypsin deficiency (AATD) is associated with chronic

149 Alpha-1 antitrypsin deficiency (AATD) liver disease is character

150 of the local folding environment in alpha-1-antitrypsin deficiency (AATD), Niemann-Pick type C1 dise

151 believed to cause lung destruction in alpha1-antitrypsin deficiency (AATD).

152 monary disease (COPD) associated with alpha1-antitrypsin deficiency (AATD).

153 alpha1-Antitrypsin deficiency (ATD) is a common genetic disorde

154 In the classical form of alpha1-antitrypsin deficiency (ATD), aberrant intracellular acc

155 y in patients with the classical form alpha1-antitrypsin deficiency (ATD).

156 es in 13 countries if they had severe alpha1 antitrypsin deficiency (serum concentration <11 muM) wit

157 Severe alpha1-antitrypsin deficiency (typically PiZZ homozygosity) is

158 Organoids from alpha1-antitrypsin deficiency and Alagille syndrome patients mi

159 ited to Mendelian syndromes, such as alpha-1 antitrypsin deficiency and cutis laxa, caused by rare ge

160 enetic and nongenetic modifiers in ZZ alpha1-antitrypsin deficiency and other disorders of protein mi

161 The conference was titled "Alpha-1-Antitrypsin Deficiency and Other Liver Diseases Caused b

162 d-stage liver disease associated with alpha1-antitrypsin deficiency and underscore the contribution o

163 disease, genetic hemochromatosis and alpha-1 antitrypsin deficiency as we continue to elucidate the m

164 Severe alpha1-antitrypsin deficiency caused by the Z variant (Glu342Ly

165 ce is comparable to the frequency of alpha-1 antitrypsin deficiency documented in this population.

166 ive measure of disease progression in alpha1 antitrypsin deficiency emphysema than spirometry is, so

167 tor (A1PI) augmentation treatment for alpha1 antitrypsin deficiency has not been substantiated by a r

168 a progression in patients with severe alpha1 antitrypsin deficiency in a randomised controlled trial

169 tic correction of a mutation causing alpha-1 antitrypsin deficiency in patient-derived hPSCs.

170 Alpha-1-antitrypsin deficiency is a genetic condition associated

171 Alpha1-antitrypsin deficiency is a genetic disease that can aff

172 million individuals worldwide, where alpha-1-antitrypsin deficiency is a major genetic cause of the d

173 Antitrypsin deficiency is a primary cause of juvenile li

174 alpha1-Antitrypsin deficiency is an inherited condition that ca

175 alpha1-Antitrypsin deficiency is one of the most common heritab

176 Alpha-1 antitrypsin deficiency shows that rare coding variants o

177 levels of which are associated with alpha-1 antitrypsin deficiency which leads to liver disease.

178 om mutations in the genes SERPINA1 (alpha(1)-antitrypsin deficiency), JAG1 (Alagille syndrome), ATP8B

179 , amyotrophic lateral sclerosis, and alpha-1 antitrypsin deficiency).

180 re LTx could be analysed (COPD, 360; alpha-1-antitrypsin deficiency, 127; interstitial lung disease,

181 In the classical form of alpha(1)-antitrypsin deficiency, a mutant protein accumulates in

182 relevant PiZZ mutation, which causes alpha1-antitrypsin deficiency, and editing of phosphotyrosine 7

183 ulated in livers from patients with alpha(1)-antitrypsin deficiency, and the degree of up-regulation

184 ndividuals in the United States have alpha-1 antitrypsin deficiency, and the most common cause of thi

185 ommon causes include hemochromatosis, alpha1-antitrypsin deficiency, autoimmune hepatitis, and Wilson

186 conditions studied in further detail (alpha1-antitrypsin deficiency, familial hypercholesterolemia, a

187 In alpha-1 antitrypsin deficiency, hepatocytes accumulate defective

188 compared data of patients with COPD, alpha-1-antitrypsin deficiency, interstitial lung disease, or cy

189 cts of multiple SERPINA1 variants on alpha-1 antitrypsin deficiency, lung function, and emphysema, th

190 ition to Mendelian syndromes such as alpha-1 antitrypsin deficiency, many genomic regions that influe

191 form of "ER stress" that occurs in alpha(1)-antitrypsin deficiency, presumably determined by the agg

192 Yet, aside from alpha-1 antitrypsin deficiency, the genetic determinants of emph

193 ar (type IV) Ehlers-Danlos syndrome, alpha-1 antitrypsin deficiency, tuberous sclerosis complex/lymph

194 al of A1PI treatment in patients with alpha1 antitrypsin deficiency.

195 uses a liver and lung disease called alpha-1 antitrypsin deficiency.

196 ases such as bacterial infection and alpha-1 antitrypsin deficiency.

197 PINA1) gene that is responsible for alpha(1)-antitrypsin deficiency.

198 ucher disease, cystic fibrosis and ZZ alpha1-antitrypsin deficiency.

199 rophil elastase for the treatment of alpha-1 antitrypsin deficiency.

200 derlies misfolding diseases such as alpha(1)-antitrypsin deficiency.

201 iseases such as cystic fibrosis, and Alpha-1 antitrypsin deficiency.

202 noncoding gene regions may result in alpha-1-antitrypsin deficiency.

203 underlies misfolding diseases such as alpha1-antitrypsin deficiency.

204 ls with emphysema secondary to severe alpha1 antitrypsin deficiency.

205 ces of emphysema distribution in non-alpha-1 antitrypsin-deficient smokers.

206 The 2.2 A structure of Thr114Phe alpha(1)-antitrypsin demonstrates that the effects of the mutatio

207 he designed tetrapeptides is the most potent antitrypsin depolymerizer yet found.

208 polymers formed by Z and His334Asp alpha(1)-antitrypsin despite the mutations directing their effect

209 matosis and iron overload disorders, alpha-1 antitrypsin disease, and exciting new therapeutic option

210 Mutant Z alpha1-antitrypsin (E342K) accumulates as polymers within the e

211 CD3delta and misfolded Z variant of alpha-1-antitrypsin, established substrates of gp78.

212 connected to the main ER network in Z-alpha1-antitrypsin-expressing cells.

213 cies in reporter assays and improves alpha-1-antitrypsin expression prediction in primary human tissu

214 ranscriptional regulatory program of alpha-1-antitrypsin expression.

215 his regulation ultimately determines alpha-1-antitrypsin expression.

216 acing the RCL sequence with that from alpha1-antitrypsin fails to restore specificity against trypsin

217 Z and shutter domain mutants of alpha(1)-antitrypsin form polymers with a shared epitope and so a

218 The alpha-1-antitrypsin gene, SERPINA1, expresses an exceptional num

219 The Z mutant of alpha1-antitrypsin (Glu342Lys) causes a domain swap and the for

220 Human albumin, alpha(1) -antitrypsin, glypican-3, alpha-smooth muscle actin, and

221 ons such as emphysema caused by human alpha1 antitrypsin (hAAT) deficiency.

222 Human alpha1-antitrypsin (hAAT) is an antiinflammatory, immune-modula

223 hepatocytes were derived from human alpha(1)-antitrypsin (hAAT) transgenic mice of the FVB strain.

224 Third, a mutant allele of human alpha1-antitrypsin (hAAT) was linked to Fah and resulted in pro

225 .56 [95% CI: -0.86, -0.26]); whereas alpha-1-antitrypsin had a negative association with HAZ (-0.28 [

226 oding variants associated with lower alpha-1 antitrypsin had greater computed tomography-based emphys

227 roteins (lactalbumin, lactoferrin, lysozyme, antitrypsin, IgA, and osteopontin).

228 ved, such as aggregation of misfolded alpha1-antitrypsin in the endoplasmic reticulum, deficient LDL

229 ulation of the misfolded Z variant of alpha1-antitrypsin in the hepatocyte endoplasmic reticulum (ER)

230 tations increase the secretion of Z alpha(1)-antitrypsin in the native conformation, but the double m

231 polymerisation of wild-type native alpha(1)-antitrypsin in vitro and increase secretion in a Xenopus

232 best described for the Z variant of alpha(1)-antitrypsin in which the proinflammatory properties of p

233 Genetic variants other than in alpha-1 antitrypsin increase the risk of COPD.

234 alpha1-Antitrypsin is a serine protease inhibitor produced in t

235 alpha(1)-Antitrypsin is a serine protease inhibitor secreted by h

236 Overexpression of Z alpha1-antitrypsin is known to induce polymer formation, prime

237 Alpha(1)-antitrypsin is the most abundant circulating protease in

238 tation therapy with intravenous AAT (alpha-1 antitrypsin) is the only specific therapy for individual

239 l shutter domain mutant (His334Asp; alpha(1)-antitrypsin King's) identified in a 6-week-old boy who p

240 eroxidase, neopterin), permeability (alpha-1-antitrypsin, lactulose, mannitol), and repair (regenerat

241 , whereas low levels of circulating Z alpha1-antitrypsin lead to emphysema by loss of inhibition of n

242 C and cathepsin Z in liver lysates or alpha1-antitrypsin levels in plasma.

243 AV vectors expressing normal, M-type alpha-1 antitrypsin (M-AAT) to AAT-deficient subjects at various

244 nated cells expressing liver-specific alpha1-antitrypsin messenger RNA, albumin and hepatocyte nuclea

245 poE, apoF, apoH, apoJ, apoL-1, apoM, alpha-1 antitrypsin, migration inhibitory factor-related protein

246 nophore, or when a nonpolymerogenic alpha(1)-antitrypsin mutant accumulated in the ER.

247 alpha-synuclein, PolyQ protein, and alpha-1-antitrypsin mutant protein.

248 effective at ratios of compound to Z alpha1-antitrypsin of 2.5:1 and reduced the intracellular accum

249 ed mice via transgenic expression of alpha-1-antitrypsin or IL-37 preserved the function of B cell pr

250 rs, such as trypsin inhibitor, serum alpha-1 antitrypsin, or liver aprotinin, are a class of proteins

251 yloid burden--c-peptide, fibrinogen, alpha-1-antitrypsin, pancreatic polypeptide, complement C3, vitr

252 variant of the gene that encodes the alpha-1 antitrypsin peptide (AAT), called the Pi*ZZ genotype, ca

253 level structural information on the alpha(1)-antitrypsin polymer.

254 eration of an mAb (4B12) that blocked alpha1-antitrypsin polymerization in vitro at a 1:1 molar ratio

255 de Maat et al on the use of altered alpha-1-antitrypsin propose therapeutic uses of these serpins fo

256 of the TGF-beta signaling pathway and alpha1-antitrypsin protein (a serine protease inhibitor) expres

257 se, inefficient secretion of a mutant alpha1-antitrypsin protein (AAT-Z) results in its accumulation

258 uORF-dependent changes suggest that alpha-1-antitrypsin protein expression levels are controlled at

259 regions in neuroserpin relative to alpha(1)-antitrypsin provides a basis for neuroserpin's increased

260 His334Asp alpha(1)-antitrypsin rapidly forms polymers that accumulate withi

261 The common Z mutant (Glu342Lys) of alpha(1)-antitrypsin results in the formation of polymers that ar

262 eins including Factor-VII[rs555212], Alpha-1-Antitrypsin[rs11846959], Interferon-Gamma Induced Protei

263 irculating bioactive peptide from the alpha1-antitrypsin serine protease inhibitor.

264 with HLA-DP and the genes encoding alpha(1)-antitrypsin (SERPINA1) and proteinase 3 (PRTN3) (P=6.2x1

265 apolipoprotein A-1 [APOA1], 3.2-fold; alpha1-antitrypsin [SERPINA1], 2.5-fold; and complement C3 [C3]

266 riant genotypes in the gene encoding alpha-1 antitrypsin, SERPINA1 (serpin peptidase inhibitor, clade

267 ining an immobile matrix of polymeric alpha1-antitrypsin, small ER resident proteins can diffuse free

268 nology to identify interactors with Z alpha1-antitrypsin that comply with both requirements.

269 cy, hepatocytes accumulate defective alpha-1 antitrypsin that misfolds.

270 peptide corresponding to a portion of alpha1-antitrypsin that potently inhibits entry of HIV-1 into h

271 ody also increased the secretion of Z alpha1-antitrypsin that retained inhibitory activity against ne

272 created fusion gene of exendin-4 and alpha1-antitrypsin to control obesity and obesity-associated me

273 and the high risk of patients lacking alpha1-antitrypsin to develop emphysema, much interest has focu

274 spose the serine protease inhibitor alpha(1)-antitrypsin to misfolding and polymerisation within hepa

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

276 ltiply charged states at m/z 72,160 ([alpha1-antitrypsin + trypsin + H](+)) and 86,585 ([IgG + protei

277 or the detection of several proteins (alpha1-antitrypsin, trypsin, IgG, protein G) and their complexe

278 on of weak protein complexes, such as alpha1-antitrypsin-trypsin and IgG-protein G complexes, at the

279 ([IgG + protein G + 2H](2+)) for the alpha1-antitrypsin-trypsin and IgG-protein G complexes, respect

280 by the exosomes from inactivation by alpha1 antitrypsin, ultimately causing the pathological degrada

281 t which N-linked glycans of misfolded alpha1-antitrypsin variant NHK were trimmed.

282 d by the instability of recombinant alpha(1)-antitrypsin variants in solution.

283 fications, affecting Lys292 in mouse alpha-1-antitrypsin, was detected in the corresponding lysine of

284 -latent transition of another serpin, alpha1-antitrypsin, which does not readily go latent.

285 est pathogenic gene mutation yields Z-alpha1-antitrypsin, which has a propensity to self-associate fo

286 YP2E1) by measuring the expression of alpha1-antitrypsin, which is controlled by these promoters and

287 Unlike other serpins such as alpha(1)-antitrypsin, wild-type neuroserpin will polymerize under

288 Wild-type alpha(1)-antitrypsin will form polymers upon incubation at modera

289 A variant of alpha(1)-antitrypsin with an E342K (Z) mutation (ATZ) has propens

290 luble secretory proteins (albumin and alpha1-antitrypsin) with that of supramolecular cargoes (e.g.,

291 polymers underlies the retention of alpha(1)-antitrypsin within hepatocytes and of neuroserpin within

292 ular accumulation of misfolded mutant alpha1-antitrypsin Z (ATZ) in hepatocytes causes hepatic damage

293 terized by accumulation of the mutant alpha1-antitrypsin Z (ATZ) variant inside cells, causing hepati

294 tracellular accumulation of misfolded alpha1-antitrypsin Z in respiratory epithelial cells of the PiZ

295 ted that the accumulation of mutant alpha(1)-antitrypsin Z in the ER specifically activates the autop

296 ers affecting the accumulation of the alpha1-antitrypsin Z mutant (ATZ) in a Caenorhabditis elegans m

297 ith the hepatic levels of insoluble alpha(1)-antitrypsin Z protein.

298 enic for the common misfolded variant alpha1-antitrypsin Z, is a model of respiratory epithelial cell

299 tical role in disposal of insoluble alpha(1)-antitrypsin Z.

300 ause of this disease is homozygosity for the antitrypsin-Z variant (ATZ).