ライフサイエンスコーパス: alpha(1) antitrypsin

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

2 nation and proteasomal degradation of mutant alpha1-antitrypsin.

3 emphysema caused by mutations in the serpin alpha1-antitrypsin.

4 ned significant amounts of human albumin and alpha1-antitrypsin.

5 bsence of polarity, and reduced secretion of alpha1-antitrypsin.

6 iver disease associated with the Z allele of alpha1-antitrypsin.

7 albumin, transferrin, alpha-fetoprotein, and alpha1-antitrypsin.

8 used by reduced level or loss of function of alpha1-antitrypsin.

9 -14, and cathepsin B and increased levels of alpha1-antitrypsin.

10 of repopulating liver cells expressing human alpha1-antitrypsin.

11 hepsins-K, -L, and -S) and the inhibition of alpha1-antitrypsin.

12 two GVHD severity markers, calprotectin and alpha1-antitrypsin.

13 , and -S and the ability of IL-13 to inhibit alpha1-antitrypsin.

14 fter its fragmentation in cells expressing Z-alpha1-antitrypsin.

15 ut compromising the inhibitory activity of Z alpha1-antitrypsin.

16 a loss of anti-inflammatory signalling by M alpha1-antitrypsin.

17 , and this could be inhibited by addition of alpha1-antitrypsin.

18 it remains stable at approximately 3.5 A in alpha(1)-antitrypsin.

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

20 h region and in beta-strand 1C compared with alpha(1)-antitrypsin.

21 oserpin while no such movement is evident in alpha(1)-antitrypsin.

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

23 d secretion when compared to the wild-type M alpha(1)-antitrypsin.

24 diate their effects on the shutter region of alpha(1)-antitrypsin.

25 cy of the key anti-elastase within the lung: alpha(1)-antitrypsin.

26 e immune response and is homologous to human alpha(1)-antitrypsin.

27 heet in heparin-complexed antithrombin or in alpha(1)-antitrypsin.

28 rences between the pathogenic Z and normal M alpha(1)-antitrypsin.

29 be partially inserted into beta-sheet A in Z alpha(1)-antitrypsin.

30 (FLEAIG) that selectively and stably bound Z alpha(1)-antitrypsin.

31 the major target of inhibition of the serpin alpha(1)-antitrypsin.

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

33 ction of a point mutation (Glu342Lys) in the alpha(1)-antitrypsin (A1AT, also known as SERPINA1) gene

34 bly, upon transplantation, human albumin and alpha1-antitrypsin (A1AT) in mouse sera secreted by enca

35 t expression of the human protease inhibitor alpha1-antitrypsin (A1AT) in Nicotiana benthamiana.

36 alpha1-Antitrypsin (A1AT) purified from human plasma upr

37 This study shows that human alpha1-antitrypsin (A1AT) upregulates expression and rel

38 alpha1-Antitrypsin (A1AT) was identified as a plasma pro

39 alpha(1)-Antitrypsin (AAT) deficiency is an underrecogni

40 hology associated with another serpinopathy, alpha(1)-antitrypsin (AAT) deficiency.

41 alpha(1)-Antitrypsin (AAT) encoded by the SERPINA1 gene

42 alpha1 -Antitrypsin (AAT) deficiency is one of the most

43 The rationale of alpha1-antitrypsin (AAT) augmentation therapy to treat p

44 Mutations in alpha1-antitrypsin (AAT) can cause the protein to polyme

45 alpha1-Antitrypsin (AAT) deficiency predisposes to bronc

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

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

48 alpha1-Antitrypsin (AAT) is a serpin, the primary functi

49 The serum protease inhibitor alpha1-antitrypsin (AAT) possesses antiinflammatory prop

50 istration of the serine proteinase inhibitor alpha1-antitrypsin (AAT) prevents type 1 diabetes develo

51 alpha1-antitrypsin (AAT) regulates the activity of multi

52 uble/insoluble distribution of two misfolded alpha1-antitrypsin (AAT) variants responsible for AAT de

53 We demonstrate that treatment with alpha1-antitrypsin (AAT), an agent that dampens inflamma

54 s were selected from the proteomic analysis, alpha1-antitrypsin (AAT), hemopexin (HX), and gelsolin (

55 g misfolded N-glycosylated variants of human alpha1-antitrypsin (AAT), Null Hong Kong (NHK), and Z (A

56 For newly synthesized alpha1-antitrypsin (AAT), the modification of its aspara

57 A sulfated alpha1-antitrypsin (AAT), thought to be a default secret

58 -acidglycoprotein) and type II (haptoglobin, alpha1-antitrypsin) acute phase proteins.

59 as used to assess the digestive stability of alpha(1)-antitrypsin against pepsin and pancreatin.

60 Polymers of Z alpha(1)-antitrypsin aggregate within the liver leading

61 nt than occurs by passive diffusion of human alpha1-antitrypsin alone.

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

63 probe the mechanism of peptide modulation of alpha(1)-antitrypsin (alpha(1)-AT) polymerization and de

64 Because retention of mutant alpha(1)-antitrypsin (alpha(1)-AT) Z in the endoplasmic

65 ecific folding of the canonical serpin human alpha(1)-antitrypsin (alpha(1)-AT).

66 rrhosis and emphysema caused by mutations in alpha(1)-antitrypsin (alpha(1)AT), and thrombosis caused

67 conformational dynamics of the serpin human alpha(1)-antitrypsin (alpha(1)AT).

68 rs to the liver of an animal model for human alpha1-antitrypsin (alpha1-AT) deficiency.

69 alpha1-Antitrypsin (alpha1-AT) is a serum protease inhib

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

71 pots specific to liver proteins: albumin and alpha1-antitrypsin (alpha1-AT).

72 In alpha(1)-antitrypsin (alpha1AT) deficiency, a polymeroge

73 Point mutants of alpha1 -antitrypsin (alpha1AT) form ordered polymers tha

74 alpha1-Antitrypsin (alpha1AT) deficiency (alpha1ATD) is

75 Although alpha1-antitrypsin (alpha1AT) does not naturally inhibit

76 s control region (LCR) upstream of the human alpha1-antitrypsin (alpha1AT) gene that is required for

77 Inhibitory activity of alpha1-antitrypsin (alpha1AT) toward elastase showed neg

78 (Glu342Lys) in the serine protease inhibitor alpha1-antitrypsin (alpha1AT), which is found in more th

79 ing oriented in a configuration resistant to alpha1-antitrypsin (alpha1AT).

80 serpin family: protein C inhibitor (PCI) and alpha1-antitrypsin (alpha1AT); however, both exhibit poo

81 ing of a known ERAD substrate, the Z form of alpha1-antitrypsin (alpha1AT-Z).

82 production could be recovered by addition of alpha1-antitrypsin, an endogenous inhibitor of serine pr

83 The protease inhibitors alpha(1)-antitrypsin and antichymotrypsin are present in

84 The results suggest that alpha(1)-antitrypsin and antichymotrypsin are produced b

85 alpha(1)-Antitrypsin and antichymotrypsin concentrations

86 Alpha(1)-antitrypsin and antichymotrypsin concentrations

87 It has been hypothesized that alpha(1)-antitrypsin and antichymotrypsin may modulate d

88 )-antitrypsin and antichymotrypsin, measured alpha(1)-antitrypsin and antichymotrypsin throughout lac

89 termined whether the mammary gland expresses alpha(1)-antitrypsin and antichymotrypsin, measured alph

90 olymerase chain reaction to detect genes for alpha(1)-antitrypsin and antichymotrypsin.

91 We show here that monomers of plasma serpins alpha(1)-antitrypsin and antithrombin are stable on incu

92 he peptide prevented the polymerization of Z alpha(1)-antitrypsin and did not significantly anneal to

93 17Phe mutations stabilise the native fold of alpha(1)-antitrypsin and increase secretion of monomeric

94 tease inhibitor family of proteins including alpha(1)-antitrypsin and protein C inhibitor.

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

96 he degradation of two other ERAD substrates, alpha1-antitrypsin and deltaCD3.

97 diazole) was used to label peroxide-modified alpha1-antitrypsin and demonstrate that the Cys-232 in v

98 distended, with significant accumulation of alpha1-antitrypsin and GRP78.

99 se had measurements of fecal lactoferrin and alpha1-antitrypsin and underwent pouch endoscopy with bi

100 correlate well with immunological levels of alpha1-antitrypsin and, thus, may prove useful for asses

101 f two serine protease inhibitors [Serpina1a (alpha1-antitrypsin) and Elafin] was dysregulated in Fbln

102 enteropathy (calprotectin, myeloperoxidase, alpha1-antitrypsin) and the prevalence of bacterial but

103 sponse genes such as SERPINA1, which encodes alpha1 antitrypsin, and FOXP4, an inhibitor of mucus pro

104 itors of metalloproteinase 2, -3, and -4 and alpha1-antitrypsin, and fibrosis was associated with inc

105 R1, TNFR2, Bid), optimal IL-13 inhibition of alpha1-antitrypsin, and IL-13-induction of and activatio

106 molecules, the solubility of mutant forms of alpha1-antitrypsin, and interactions with newly synthesi

107 oembryonic antigen, retinol binding protein, alpha1-antitrypsin, and squamous cell carcinoma antigen-

108 termined levels of carcinoembryonic antigen, alpha1-antitrypsin, and squamous cell carcinoma antigen.

109 Serum levels of nine biomarkers (alpha1 antitrypsin, apolipoprotein CIII, brain-derived n

110 The S- and Z-deficiency alleles of alpha1-antitrypsin are found in more than 20% of some wh

111 tracellular serpins such as antithrombin and alpha1-antitrypsin are the quintessential regulators of

112 ave assessed a surface hydrophobic cavity in alpha1-antitrypsin as a potential target for rational dr

113 ts identifying cathepsin C, cathepsin Z, and alpha1-antitrypsin as additional potential cargoes for L

114 R spectroscopy to patient-derived samples of alpha(1)-antitrypsin at natural isotopic abundance to in

115 y, and levels of inflammatory biomarkers and alpha1-antitrypsin at baseline.

116 Alpha(1)-antitrypsin (AT) is the most abundantly circula

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

118 hial epithelial cells with purified plasma M alpha1-antitrypsin attenuates this inflammatory response

119 echanism due to accumulation of the mutant Z alpha1-antitrypsin (ATZ) and is a key example of an dise

120 alpha(1)-Antitrypsin blocked FNf-induced shedding of CD4

121 nzymes retain vulnerability to inhibition by alpha(1)-antitrypsin, but demonstrate variable avidity f

122 Replacement of His-334 in alpha(1)-antitrypsin by a serine or alanine at pH 7.4 re

123 mutation reduces concentrations in serum of alpha1 antitrypsin by retaining polymerised molecules wi

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

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

126 biomarkers, followed by IL-2 receptor alpha, alpha1-antitrypsin, C-reactive protein, YKL-40, cellular

127 inhibited by alpha(1)-proteinase inhibitor (alpha(1)-antitrypsin), C1 inhibitor, and most efficientl

128 Polymers of mutant alpha1-antitrypsin can also form within the alveoli and

129 ility of the GeneSwitch, we cloned the human alpha(1)-antitrypsin cDNA into the optimal lentiviral ve

130 f interleukin-6, interleukin-8, and elastase-alpha1-antitrypsin complexes compared with presurgery le

131 f interleukin-8, interleukin-6, and elastase-alpha1-antitrypsin complexes were elevated compared with

132 ls of interleukin-6, interleukin-8, elastase-alpha1-antitrypsin complexes, thrombin-antithrombin comp

133 Treatment with the serine protease inhibitor alpha1-antitrypsin decreased serum levels of HS, leading

134 Alpha1-antitrypsin defciency-related liver disease is th

135 alpha(1)-Antitrypsin deficiency (AATD) is an inherited d

136 result from mutations in the genes SERPINA1 (alpha(1)-antitrypsin deficiency), JAG1 (Alagille syndrom

137 In the classical form of alpha(1)-antitrypsin deficiency, a mutant protein accumu

138 as up-regulated in livers from patients with alpha(1)-antitrypsin deficiency, and the degree of up-re

139 The polymerization of AT, leading to alpha(1)-antitrypsin deficiency, has been studied extens

140 distinct form of "ER stress" that occurs in alpha(1)-antitrypsin deficiency, presumably determined b

141 wn as SERPINA1) gene that is responsible for alpha(1)-antitrypsin deficiency.

142 n, and underlies misfolding diseases such as alpha(1)-antitrypsin deficiency.

143 6 male and 4 female former smokers, two with alpha(1)-antitrypsin deficiency.

144 rs that can be used to treat patients with Z alpha(1)-antitrypsin deficiency.

145 y centres in 13 countries if they had severe alpha1 antitrypsin deficiency (serum concentration <11 m

146 sensitive measure of disease progression in alpha1 antitrypsin deficiency emphysema than spirometry

147 inhibitor (A1PI) augmentation treatment for alpha1 antitrypsin deficiency has not been substantiated

148 mphysema progression in patients with severe alpha1 antitrypsin deficiency in a randomised controlled

149 dividuals with emphysema secondary to severe alpha1 antitrypsin deficiency.

150 led trial of A1PI treatment in patients with alpha1 antitrypsin deficiency.

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

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

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

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

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

156 verity and distribution in 119 subjects with alpha1-antitrypsin deficiency (PiZ phenotype) and groupe

157 Severe alpha1-antitrypsin deficiency (typically PiZZ homozygosi

158 Organoids from alpha1-antitrypsin deficiency and Alagille syndrome pati

159 ng of genetic and nongenetic modifiers in ZZ alpha1-antitrypsin deficiency and other disorders of pro

160 the end-stage liver disease associated with alpha1-antitrypsin deficiency and underscore the contrib

161 he most frequent mutation that causes severe alpha1-antitrypsin deficiency arises in the SERPINA 1 ge

162 Severe alpha1-antitrypsin deficiency caused by the Z variant (G

163 ents with CF, primary ciliary dyskinesia, or alpha1-antitrypsin deficiency exhibited 3-fold higher mu

164 netic hemochromatosis, Wilson's disease, and alpha1-antitrypsin deficiency grow significantly.

165 Alpha1-antitrypsin deficiency is a genetic disease that

166 Alpha1-antitrypsin deficiency is a genetic disorder that

167 alpha1-Antitrypsin deficiency is an inherited condition

168 alpha1-Antitrypsin deficiency is one of the most common

169 The association of alpha1-antitrypsin deficiency with the development of em

170 nically relevant PiZZ mutation, which causes alpha1-antitrypsin deficiency, and editing of phosphotyr

171 Less common causes include hemochromatosis, alpha1-antitrypsin deficiency, autoimmune hepatitis, and

172 ess of augmentation therapy (Aug) for severe alpha1-antitrypsin deficiency, comparing strategies of:

173 abolic conditions studied in further detail (alpha1-antitrypsin deficiency, familial hypercholesterol

174 In alpha1-antitrypsin deficiency, intrahepatocyte accumulat

175 ding Gaucher disease, cystic fibrosis and ZZ alpha1-antitrypsin deficiency.

176 that underlies emphysema in individuals with alpha1-antitrypsin deficiency.

177 stemic inflammatory diseases associated with alpha1-antitrypsin deficiency.

178 apy for treatment of liver diseases, such as alpha1-antitrypsin deficiency.

179 tions in CF, primary ciliary dyskinesia, and alpha1-antitrypsin deficiency.

180 clinically and cost-effective therapies for alpha1-antitrypsin deficiency.

181 which underlies misfolding diseases such as alpha1-antitrypsin deficiency.

182 re, early-onset COPD probands without severe alpha1-antitrypsin deficiency.

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

184 ecognizes polymers formed by Z and His334Asp alpha(1)-antitrypsin despite the mutations directing the

185 ther hereditary iron overload disorders, and alpha1-antitrypsin disease-are the focus of this review.

186 se diseases are typified by the Z variant of alpha(1)-antitrypsin (E342K), which causes the retention

187 Mutant Z alpha1-antitrypsin (E342K) accumulates as polymers withi

188 equence corresponding to residues 359-374 of alpha1-antitrypsin, enhances gene expression from DNA na

189 ns are connected to the main ER network in Z-alpha1-antitrypsin-expressing cells.

190 Replacing the RCL sequence with that from alpha1-antitrypsin fails to restore specificity against

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

192 are identical to the Z-deficiency variant of alpha(1)-antitrypsin form urea-stable polymers in vivo.

193 eatments for emphysema, infusion of purified alpha1 antitrypsin from pooled human plasma represents a

194 of an 8-kb DNA segment upstream of the human alpha1-antitrypsin gene yields a mutant serpin allele th

195 The Z mutant of alpha1-antitrypsin (Glu342Lys) causes a domain swap and

196 Human albumin, alpha(1) -antitrypsin, glypican-3, alpha-smooth muscle a

197 Donor hepatocytes were derived from human alpha(1)-antitrypsin (hAAT) transgenic mice of the FVB s

198 mutations such as emphysema caused by human alpha1 antitrypsin (hAAT) deficiency.

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

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

201 gents, monotherapy with clinical-grade human alpha1-antitrypsin (hAAT), the major serum serine-protea

202 eactive protein, alpha(1)-acid glycoprotein, alpha(1)-antitrypsin, haptoglobin, and fibrinogen concen

203 e fractional and absolute synthesis rates of alpha(1)-antitrypsin, haptoglobin, and fibrinogen were m

204 The Z mutant of alpha(1)-antitrypsin has a point mutation Glu342Lys in t

205 Both mutations increase the secretion of Z alpha(1)-antitrypsin in the native conformation, but the

206 educe the polymerisation of wild-type native alpha(1)-antitrypsin in vitro and increase secretion in

207 pidly inactivated by the human plasma serpin alpha(1)-antitrypsin in vitro, administration of recombi

208 This is best described for the Z variant of alpha(1)-antitrypsin in which the proinflammatory proper

209 denoassociated virus vector expressing human alpha1-antitrypsin in murine liver progenitor cells.

210 re derived, such as aggregation of misfolded alpha1-antitrypsin in the endoplasmic reticulum, deficie

211 e accumulation of the misfolded Z variant of alpha1-antitrypsin in the hepatocyte endoplasmic reticul

212 obtained in SU5416-treated rats given human alpha1-antitrypsin intravenously.

213 alpha(1)-Antitrypsin is a serine protease inhibitor secr

214 Alpha(1)-antitrypsin is the most abundant circulating pr

215 Although alpha1 antitrypsin is mainly produced in the liver, its

216 alpha1-Antitrypsin is a serine protease inhibitor produc

217 findings have indicated that a deficiency in alpha1-antitrypsin is associated with increased risk of

218 ellular portion of the pIgR, linked to human alpha1-antitrypsin is effectively ferried across human t

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

220 nd a novel shutter domain mutant (His334Asp; alpha(1)-antitrypsin King's) identified in a 6-week-old

221 disease, whereas low levels of circulating Z alpha1-antitrypsin lead to emphysema by loss of inhibiti

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

223 alpha(1)-Antitrypsin may survive digestion and may affec

224 cal production of polymers by mutant S and Z alpha1-antitrypsin may have also provided protection aga

225 in addition to its antielastolytic effects, alpha1-antitrypsin may have broader biological effects i

226 n-originated cells expressing liver-specific alpha1-antitrypsin messenger RNA, albumin and hepatocyte

227 lt in a conformational transition within the alpha1-antitrypsin molecule and the formation of polymer

228 alcium ionophore, or when a nonpolymerogenic alpha(1)-antitrypsin mutant accumulated in the ER.

229 se protective, proinflammatory properties of alpha1-antitrypsin mutants have become detrimental to ca

230 y, intrahepatocyte accumulation of defective alpha(1)-antitrypsin occurs.

231 ts were effective at ratios of compound to Z alpha1-antitrypsin of 2.5:1 and reduced the intracellula

232 helium protease is not highly susceptible to alpha1-antitrypsin or secretory leukocyte protease inhib

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

234 the generation of an mAb (4B12) that blocked alpha1-antitrypsin polymerization in vitro at a 1:1 mola

235 substrate decanoyl-RVKR-chloromethylketone, alpha1-antitrypsin Portland and by its own propeptide.

236 study, we show that inducible expression of alpha1-antitrypsin Portland, a furin inhibitor, inhibits

237 Lactoferrin, with alpha(1)-antitrypsin present, was digested by pancreatin

238 ific promoter (murine albumin enhancer/human alpha1-antitrypsin promoter) further enhanced transgene

239 tutively active FoxO1 in the liver using the alpha1-antitrypsin promoter.

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

241 e disease, inefficient secretion of a mutant alpha1-antitrypsin protein (AAT-Z) results in its accumu

242 of these regions in neuroserpin relative to alpha(1)-antitrypsin provides a basis for neuroserpin's

243 His334Asp alpha(1)-antitrypsin rapidly forms polymers that accumul

244 ummary, this work provides new insights into alpha1-antitrypsin reactivity in oxidizing environments

245 rophils in the alveoli of individuals with Z alpha(1)-antitrypsin-related emphysema.

246 ssue destruction that is characteristic of Z alpha(1)-antitrypsin-related emphysema.

247 ion of emphysema in some individuals despite alpha(1)-antitrypsin replacement therapy.

248 ion of structural alveolar cell apoptosis by alpha1-antitrypsin represents a novel protective mechani

249 The common Z mutant (Glu342Lys) of alpha(1)-antitrypsin results in the formation of polymer

250 de, a circulating bioactive peptide from the alpha1-antitrypsin serine protease inhibitor.

251 ssociated with HLA-DP and the genes encoding alpha(1)-antitrypsin (SERPINA1) and proteinase 3 (PRTN3)

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

253 s containing an immobile matrix of polymeric alpha1-antitrypsin, small ER resident proteins can diffu

254 Ab technology to identify interactors with Z alpha1-antitrypsin that comply with both requirements.

255 tify a peptide corresponding to a portion of alpha1-antitrypsin that potently inhibits entry of HIV-1

256 intrabody also increased the secretion of Z alpha1-antitrypsin that retained inhibitory activity aga

257 Relative to alpha(1)-antitrypsin, the reactive site loop of AT has t

258 but did increase the levels of mRNA encoding alpha1-antitrypsin, tissue inhibitor of metalloproteinas

259 proteolysis, and determined the potential of alpha(1)-antitrypsin to affect the survival of other mil

260 ons predispose the serine protease inhibitor alpha(1)-antitrypsin to misfolding and polymerisation wi

261 The transition of native Z alpha(1)-antitrypsin to polymers inactivates its anti-pr

262 ughout lactation, assessed the resistance of alpha(1)-antitrypsin to proteolysis, and determined the

263 a newly created fusion gene of exendin-4 and alpha1-antitrypsin to control obesity and obesity-associ

264 tissue and the high risk of patients lacking alpha1-antitrypsin to develop emphysema, much interest h

265 rved multiply charged states at m/z 72,160 ([alpha1-antitrypsin + trypsin + H](+)) and 86,585 ([IgG +

266 atrix for the detection of several proteins (alpha1-antitrypsin, trypsin, IgG, protein G) and their c

267 detection of weak protein complexes, such as alpha1-antitrypsin-trypsin and IgG-protein G complexes,

268 86,585 ([IgG + protein G + 2H](2+)) for the alpha1-antitrypsin-trypsin and IgG-protein G complexes,

269 otected by the exosomes from inactivation by alpha1 antitrypsin, ultimately causing the pathological

270 rate at which N-linked glycans of misfolded alpha1-antitrypsin variant NHK were trimmed.

271 is limited by the instability of recombinant alpha(1)-antitrypsin variants in solution.

272 NTS, AND MAIN RESULTS: Transduction of human alpha1-antitrypsin via replication-deficient adeno-assoc

273 still intact after digestion, but only when alpha(1)-antitrypsin was added.

274 This conformational difference from M alpha(1)-antitrypsin was exploited with a 6-mer reactive

275 After in vitro digestion, much of the alpha(1)-antitrypsin was still intact, whereas many othe

276 ggested that a significant fraction of their alpha(1)-antitrypsin was tied up in high molecular mass

277 Fecal alpha1-antitrypsin was not able to distinguish symptomat

278 errant form of the hepatic secretory protein alpha1-antitrypsin was stably expressed in a human embry

279 addition, transgene expression (serum human alpha1-antitrypsin) was sustained for the length of the

280 ch corresponds to Met(358), the P(1) site of alpha1-antitrypsin, was the inhibitory site for elastase

281 he P6-P1 region of the reactive site loop of alpha(1)-antitrypsin were constructed.

282 noassociated virus 1-vector-expressing human alpha1 antitrypsin were transplanted into the liver of m

283 tive-to-latent transition of another serpin, alpha1-antitrypsin, which does not readily go latent.

284 commonest pathogenic gene mutation yields Z-alpha1-antitrypsin, which has a propensity to self-assoc

285 alpha1-Antitrypsin, which is a metastable and conformati

286 2E1 (CYP2E1) by measuring the expression of alpha1-antitrypsin, which is controlled by these promote

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

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

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

290 t of soluble secretory proteins (albumin and alpha1-antitrypsin) with that of supramolecular cargoes

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

292 e to inactivation by protein C inhibitor and alpha(1)-antitrypsin yet maintained their primary antico

293 ve indicated that the accumulation of mutant alpha(1)-antitrypsin Z in the ER specifically activates

294 related with the hepatic levels of insoluble alpha(1)-antitrypsin Z protein.

295 ays a critical role in disposal of insoluble alpha(1)-antitrypsin Z.

296 tracellular accumulation of misfolded mutant alpha1-antitrypsin Z (ATZ) in hepatocytes causes hepatic

297 characterized by accumulation of the mutant alpha1-antitrypsin Z (ATZ) variant inside cells, causing

298 Intracellular accumulation of misfolded alpha1-antitrypsin Z in respiratory epithelial cells of

299 modifiers affecting the accumulation of the alpha1-antitrypsin Z mutant (ATZ) in a Caenorhabditis el

300 transgenic for the common misfolded variant alpha1-antitrypsin Z, is a model of respiratory epitheli