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1 inactivation by alpha1-proteinase inhibitor (alpha1-antitrypsin).
2 emphysema caused by mutations in the serpin alpha1-antitrypsin.
3 ned significant amounts of human albumin and alpha1-antitrypsin.
4 bsence of polarity, and reduced secretion of alpha1-antitrypsin.
5 iver disease associated with the Z allele of alpha1-antitrypsin.
6 albumin, transferrin, alpha-fetoprotein, and alpha1-antitrypsin.
7 used by reduced level or loss of function of alpha1-antitrypsin.
8 two GVHD severity markers, calprotectin and 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 , and -S and the ability of IL-13 to inhibit alpha1-antitrypsin.
13 and 2300-fold higher than that of wild-type alpha1-antitrypsin.
14 ng from mutations of the prototypical serpin alpha1-antitrypsin.
15 the result of entangled polymers of mutant Z alpha1-antitrypsin.
16 e expression, including HNF4, HNF1alpha, and alpha1-antitrypsin.
17 formation of heteropolymers between S and Z alpha1-antitrypsin.
18 d for the formation of a stable complex with alpha1-antitrypsin.
19 fter its fragmentation in cells expressing Z-alpha1-antitrypsin.
20 important component of the function of human alpha1-antitrypsin.
21 es of the APPs transferrin, haptoglobin, and alpha1-antitrypsin.
22 ut compromising the inhibitory activity of Z alpha1-antitrypsin.
23 a loss of anti-inflammatory signalling by M alpha1-antitrypsin.
24 , and this could be inhibited by addition of alpha1-antitrypsin.
25 nation and proteasomal degradation of mutant alpha1-antitrypsin.
26 another conformationally unstable variant (I alpha1-antitrypsin; 39Arg-->Cys) identified in a 34-year
28 evaluate the function of this loop, we used alpha1-antitrypsin, a non-heparin-binding serpin and slo
29 bly, upon transplantation, human albumin and alpha1-antitrypsin (A1AT) in mouse sera secreted by enca
32 grafts transduced to express a transgene for alpha1-antitrypsin (A1AT) successfully restored physiolo
40 C and alcoholism developed large numbers of alpha1-antitrypsin (AAT) globules within hepatocytes of
41 e, we analyzed the production efficiency for alpha1-antitrypsin (AAT) in Chinese hamster ovary cells,
45 istration of the serine proteinase inhibitor alpha1-antitrypsin (AAT) prevents type 1 diabetes develo
47 uble/insoluble distribution of two misfolded alpha1-antitrypsin (AAT) variants responsible for AAT de
48 mbalance between PR3 and its major inhibitor alpha1-antitrypsin (AAT) was proposed to underlie PR3- b
50 eptide derived from the carboxyl-terminus of alpha1-Antitrypsin (AAT), corresponding to residues 364-
51 s were selected from the proteomic analysis, alpha1-antitrypsin (AAT), hemopexin (HX), and gelsolin (
52 g misfolded N-glycosylated variants of human alpha1-antitrypsin (AAT), Null Hong Kong (NHK), and Z (A
55 on, kidney lysosomal enzyme elevation, serum alpha1-antitrypsin activity deficiency and abnormal otol
59 e ER of liver cells in the classical form of alpha1 antitrypsin (alpha1-AT) deficiency is associated
60 receptor initially identified for binding of alpha1-antitrypsin (alpha1-AT) and other serine protease
64 t ER degradation of mutant secretory protein alpha1-antitrypsin (alpha1-AT) Z, the mutant protein ass
66 d levels of IL-8, protein, LDH, fibronectin, alpha1-antitrypsin (alpha1-AT), complement fragment 3a (
67 e inhibitor of metalloproteinase-1 (TIMP-1), alpha1-antitrypsin (alpha1-AT), protease nexin II (PN-II
69 ription of three HNF-4alpha sensitive genes, alpha1-antitrypsin (alpha1-AT), transthyretin (TTR), and
72 are mainly expressed in the liver, including alpha1-antitrypsin, alpha1-antichymotrypsin, alpha-fetal
76 last somatic cell hybrids, extinction of rat alpha1-antitrypsin (alpha1AT) gene expression is accompa
78 s control region (LCR) upstream of the human alpha1-antitrypsin (alpha1AT) gene that is required for
82 (Glu342Lys) in the serine protease inhibitor alpha1-antitrypsin (alpha1AT), which is found in more th
83 in, heparin cofactor II (HCII)-thrombin, and alpha1-antitrypsin (alpha1AT)-trypsin bound to purified
85 serpin family: protein C inhibitor (PCI) and alpha1-antitrypsin (alpha1AT); however, both exhibit poo
91 production could be recovered by addition of alpha1-antitrypsin, an endogenous inhibitor of serine pr
93 reported here of a mixed heterozygote for Z alpha1-antitrypsin and another conformationally unstable
94 homologous in sequence and function to human alpha1-antitrypsin and are encoded by a highly conserved
96 diazole) was used to label peroxide-modified alpha1-antitrypsin and demonstrate that the Cys-232 in v
99 physical association between unfolded human alpha1-antitrypsin and UDP-glucose:glycoprotein glucosyl
100 se had measurements of fecal lactoferrin and alpha1-antitrypsin and underwent pouch endoscopy with bi
101 correlate well with immunological levels of alpha1-antitrypsin and, thus, may prove useful for asses
102 f two serine protease inhibitors [Serpina1a (alpha1-antitrypsin) and Elafin] was dysregulated in Fbln
103 enteropathy (calprotectin, myeloperoxidase, alpha1-antitrypsin) and the prevalence of bacterial but
104 sponse genes such as SERPINA1, which encodes alpha1 antitrypsin, and FOXP4, an inhibitor of mucus pro
105 itors of metalloproteinase 2, -3, and -4 and alpha1-antitrypsin, and fibrosis was associated with inc
106 R1, TNFR2, Bid), optimal IL-13 inhibition of alpha1-antitrypsin, and IL-13-induction of and activatio
107 molecules, the solubility of mutant forms of alpha1-antitrypsin, and interactions with newly synthesi
108 es) is the target for protease inhibition by alpha1-antitrypsin, and its unopposed release destroys t
109 oembryonic antigen, retinol binding protein, alpha1-antitrypsin, and squamous cell carcinoma antigen-
110 termined levels of carcinoembryonic antigen, alpha1-antitrypsin, and squamous cell carcinoma antigen.
113 tracellular serpins such as antithrombin and alpha1-antitrypsin are the quintessential regulators of
114 ave assessed a surface hydrophobic cavity in alpha1-antitrypsin as a potential target for rational dr
115 ts identifying cathepsin C, cathepsin Z, and alpha1-antitrypsin as additional potential cargoes for L
119 hial epithelial cells with purified plasma M alpha1-antitrypsin attenuates this inflammatory response
120 echanism due to accumulation of the mutant Z alpha1-antitrypsin (ATZ) and is a key example of an dise
121 C-terminal end of the serpin (e.g. FVFLM in alpha1-antitrypsin) binds to the serpin-enzyme complex r
122 (native), thrombin-antithrombin, or elastase-alpha1-antitrypsin, but not by free HCII or thrombin, wh
123 mutation reduces concentrations in serum of alpha1 antitrypsin by retaining polymerised molecules wi
125 reduced the intracellular accumulation of Z alpha1-antitrypsin by 70% in a cell model of disease.
126 loss, bleeding on probing), serum NT-proBNP, alpha1-antitrypsin, C-reactive protein (hs-CRP), endothe
127 biomarkers, followed by IL-2 receptor alpha, alpha1-antitrypsin, C-reactive protein, YKL-40, cellular
132 ent here the 2.6 A structure of a polymer of alpha1-antitrypsin cleaved six residues N-terminal to th
133 philia and plasma concentrations of elastase-alpha1-antitrypsin complexes and lactoferrin), but did n
134 f interleukin-6, interleukin-8, and elastase-alpha1-antitrypsin complexes compared with presurgery le
135 f interleukin-8, interleukin-6, and elastase-alpha1-antitrypsin complexes were elevated compared with
137 hil degranulation (plasma levels of elastase-alpha1-antitrypsin complexes, P < .05) and modestly redu
138 ls of interleukin-6, interleukin-8, elastase-alpha1-antitrypsin complexes, thrombin-antithrombin comp
139 Treatment with the serine protease inhibitor alpha1-antitrypsin decreased serum levels of HS, leading
141 y centres in 13 countries if they had severe alpha1 antitrypsin deficiency (serum concentration <11 m
142 sensitive measure of disease progression in alpha1 antitrypsin deficiency emphysema than spirometry
143 inhibitor (A1PI) augmentation treatment for alpha1 antitrypsin deficiency has not been substantiated
144 mphysema progression in patients with severe alpha1 antitrypsin deficiency in a randomised controlled
153 verity and distribution in 119 subjects with alpha1-antitrypsin deficiency (PiZ phenotype) and groupe
158 ng of genetic and nongenetic modifiers in ZZ alpha1-antitrypsin deficiency and other disorders of pro
159 the end-stage liver disease associated with alpha1-antitrypsin deficiency and underscore the contrib
160 he most frequent mutation that causes severe alpha1-antitrypsin deficiency arises in the SERPINA 1 ge
162 ents with CF, primary ciliary dyskinesia, or alpha1-antitrypsin deficiency exhibited 3-fold higher mu
164 insulin signaling in the PiZ mouse model of alpha1-antitrypsin deficiency in which hepatocellular ac
171 nically relevant PiZZ mutation, which causes alpha1-antitrypsin deficiency, and editing of phosphotyr
172 netic disorders, such as cystic fibrosis and alpha1-antitrypsin deficiency, and for other diseases, i
173 with either alcohol-related liver disease or alpha1-antitrypsin deficiency, and only one of the healt
174 Less common causes include hemochromatosis, alpha1-antitrypsin deficiency, autoimmune hepatitis, and
175 blished pulmonary mechanics in patients with alpha1-antitrypsin deficiency, chronic obstructive pulmo
176 ess of augmentation therapy (Aug) for severe alpha1-antitrypsin deficiency, comparing strategies of:
177 abolic conditions studied in further detail (alpha1-antitrypsin deficiency, familial hypercholesterol
179 ria or tuberculosis, genetic conditions (eg, alpha1-antitrypsin deficiency, primary ciliary dyskinesi
189 tients with alcohol-related liver disease or alpha1-antitrypsin-deficiency liver disease, and only on
190 ther hereditary iron overload disorders, and alpha1-antitrypsin disease-are the focus of this review.
192 equence corresponding to residues 359-374 of alpha1-antitrypsin, enhances gene expression from DNA na
194 Replacing the RCL sequence with that from alpha1-antitrypsin fails to restore specificity against
195 rects calnexin in the selection of misfolded alpha1-antitrypsin for degradation by the proteasome.
196 eatments for emphysema, infusion of purified alpha1 antitrypsin from pooled human plasma represents a
197 ccharides abrogates the release of misfolded alpha1-antitrypsin from calnexin prior to proteasomal de
200 of an 8-kb DNA segment upstream of the human alpha1-antitrypsin gene yields a mutant serpin allele th
202 A transgenic mouse line expressing the human alpha1-antitrypsin (hA1AT) gene was developed in an FVB/
203 " hepatocytes from mice transgenic for human alpha1-antitrypsin (hA1AT) were transplanted by intraspl
207 gents, monotherapy with clinical-grade human alpha1-antitrypsin (hAAT), the major serum serine-protea
208 ecreased the persistence of transgene (human alpha1-antitrypsin [hAAT]) expression that was associate
209 At 6-month follow-up, the reduced NT-proBNP, alpha1-antitrypsin, hs-CRP, ECM-1, and NGAL levels were
210 Ad) vector-encoded proteins, including human alpha1-antitrypsin (huAAT), mouse erythropoietin (EPO),
211 TGN, where it stimulates the sialylation of alpha1-antitrypsin (i.e. one of the identified secretory
212 denoassociated virus vector expressing human alpha1-antitrypsin in murine liver progenitor cells.
213 re derived, such as aggregation of misfolded alpha1-antitrypsin in the endoplasmic reticulum, deficie
214 e accumulation of the misfolded Z variant of alpha1-antitrypsin in the hepatocyte endoplasmic reticul
218 findings have indicated that a deficiency in alpha1-antitrypsin is associated with increased risk of
219 ellular portion of the pIgR, linked to human alpha1-antitrypsin is effectively ferried across human t
221 disease, whereas low levels of circulating Z alpha1-antitrypsin lead to emphysema by loss of inhibiti
223 nt human MARs, from the apolipoprotein B and alpha1-antitrypsin loci, insulated white transgene expre
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 se protective, proinflammatory properties of alpha1-antitrypsin mutants have become detrimental to ca
229 ts were effective at ratios of compound to Z alpha1-antitrypsin of 2.5:1 and reduced the intracellula
230 ex, did not alter the inactivation of APC by alpha1-antitrypsin or protein C inhibitor, and did not i
231 helium protease is not highly susceptible to alpha1-antitrypsin or secretory leukocyte protease inhib
232 f NT-proBNP (p = 0.004), hs-CRP (p = 0.003), alpha1-antitrypsin (p = 0.012), ECM-1 (p = 0.014), and N
233 The mutation in the Z deficiency variant of alpha1-antitrypsin perturbs the structure of the protein
234 es deleted and containing the complete human alpha1-antitrypsin (PI) locus, we observed tissue-specif
235 a framework for understanding the uncleaved alpha1-antitrypsin polymer and fibrillar and amyloid dep
236 allow us to propose a kinetic mechanism for alpha1-antitrypsin polymer formation that involves the g
237 the generation of an mAb (4B12) that blocked alpha1-antitrypsin polymerization in vitro at a 1:1 mola
238 iophysical techniques have demonstrated that alpha1-antitrypsin polymerization is a two-stage process
240 e the utility of the protein-based inhibitor alpha1-antitrypsin Portland (alpha1-PDX) as an antipatho
241 pressed a potent protein inhibitor of furin, alpha1-antitrypsin Portland (alpha1-PDX) in early Xenopu
242 substrate decanoyl-RVKR-chloromethylketone, alpha1-antitrypsin Portland and by its own propeptide.
243 study, we show that inducible expression of alpha1-antitrypsin Portland, a furin inhibitor, inhibits
245 ific promoter (murine albumin enhancer/human alpha1-antitrypsin promoter) further enhanced transgene
247 lation of the TGF-beta signaling pathway and alpha1-antitrypsin protein (a serine protease inhibitor)
248 e disease, inefficient secretion of a mutant alpha1-antitrypsin protein (AAT-Z) results in its accumu
249 of several tested in yielding expression of alpha1-antitrypsin protein from a retroviral vector in h
251 ummary, this work provides new insights into alpha1-antitrypsin reactivity in oxidizing environments
252 brane protein, or the soluble PiZ variant of alpha1-antitrypsin, reduced levels of general COPII vesi
253 ion of structural alveolar cell apoptosis by alpha1-antitrypsin represents a novel protective mechani
254 oyed to investigate quality control of human alpha1-antitrypsin secretion from stably transfected mou
256 -fold; apolipoprotein A-1 [APOA1], 3.2-fold; alpha1-antitrypsin [SERPINA1], 2.5-fold; and complement
257 By injection of plasmid DNA encoding human alpha1-antitrypsin, significant concentrations of hAAT w
258 s containing an immobile matrix of polymeric alpha1-antitrypsin, small ER resident proteins can diffu
259 Ab technology to identify interactors with Z alpha1-antitrypsin that comply with both requirements.
260 tify a peptide corresponding to a portion of alpha1-antitrypsin that potently inhibits entry of HIV-1
261 intrabody also increased the secretion of Z alpha1-antitrypsin that retained inhibitory activity aga
262 but did increase the levels of mRNA encoding alpha1-antitrypsin, tissue inhibitor of metalloproteinas
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 Using cytomegalovirus (CMV)-driven human alpha1-antitrypsin, transgene expression was immunolocal
266 rved multiply charged states at m/z 72,160 ([alpha1-antitrypsin + trypsin + H](+)) and 86,585 ([IgG +
267 atrix for the detection of several proteins (alpha1-antitrypsin, trypsin, IgG, protein G) and their c
268 86,585 ([IgG + protein G + 2H](2+)) for the alpha1-antitrypsin-trypsin and IgG-protein G complexes,
269 detection of weak protein complexes, such as alpha1-antitrypsin-trypsin and IgG-protein G complexes,
270 Plasma concentrations of C-reactive protein, alpha1-antitrypsin, tumor necrosis factor alpha (TNF-alp
271 otected by the exosomes from inactivation by alpha1 antitrypsin, ultimately causing the pathological
274 iants were needed to slow the degradation of alpha1-antitrypsin variant Z in cells from a protected s
275 34A could each accelerate the degradation of alpha1-antitrypsin variant Z in cells from the index pat
276 NTS, AND MAIN RESULTS: Transduction of human alpha1-antitrypsin via replication-deficient adeno-assoc
277 acellular fate of terminally misfolded human alpha1-antitrypsin was examined in hepatoma cells to ide
279 errant form of the hepatic secretory protein alpha1-antitrypsin was stably expressed in a human embry
280 addition, transgene expression (serum human alpha1-antitrypsin) was sustained for the length of the
281 ch corresponds to Met(358), the P(1) site of alpha1-antitrypsin, was the inhibitory site for elastase
282 noassociated virus 1-vector-expressing human alpha1 antitrypsin were transplanted into the liver of m
283 Escherichia coli beta-galactosidase or human alpha1-antitrypsin were prepared and expressed the repor
284 reactive loop and shutter domain mutants of alpha1-antitrypsin were used to demonstrate the close as
285 tive-to-latent transition of another serpin, alpha1-antitrypsin, which does not readily go latent.
286 commonest pathogenic gene mutation yields Z-alpha1-antitrypsin, which has a propensity to self-assoc
288 2E1 (CYP2E1) by measuring the expression of alpha1-antitrypsin, which is controlled by these promote
289 for the Z variant, but even more common is S alpha1-antitrypsin, which is found in up to 28% of south
290 serpins (antithrombin, protease nexin-1 and alpha1-antitrypsin with a P1 arginine) were 2 x 10(3) to
291 t of soluble secretory proteins (albumin and alpha1-antitrypsin) with that of supramolecular cargoes
294 tracellular accumulation of misfolded mutant alpha1-antitrypsin Z (ATZ) in hepatocytes causes hepatic
295 characterized by accumulation of the mutant alpha1-antitrypsin Z (ATZ) variant inside cells, causing
296 -antitrypsin deficiency, a misfolded variant alpha1-antitrypsin Z accumulates in the endoplasmic reti
298 modifiers affecting the accumulation of the alpha1-antitrypsin Z mutant (ATZ) in a Caenorhabditis el
299 mulation and proteotoxicity of the misfolded alpha1-antitrypsin Z variant (ATZ) causes liver fibrosis
300 transgenic for the common misfolded variant alpha1-antitrypsin Z, is a model of respiratory epitheli