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1 inactivation by alpha1-proteinase inhibitor (alpha1-antitrypsin).
2 ned significant amounts of human albumin and alpha1-antitrypsin.
3 bsence of polarity, and reduced secretion of alpha1-antitrypsin.
4 iver disease associated with the Z allele of alpha1-antitrypsin.
5 albumin, transferrin, alpha-fetoprotein, and alpha1-antitrypsin.
6 used by reduced level or loss of function of alpha1-antitrypsin.
7 -14, and cathepsin B and increased levels of alpha1-antitrypsin.
8 fter its fragmentation in cells expressing Z-alpha1-antitrypsin.
9 of repopulating liver cells expressing human alpha1-antitrypsin.
10 hepsins-K, -L, and -S) and the inhibition of alpha1-antitrypsin.
11 , and -S and the ability of IL-13 to inhibit alpha1-antitrypsin.
12 and 2300-fold higher than that of wild-type alpha1-antitrypsin.
13 ng from mutations of the prototypical serpin alpha1-antitrypsin.
14 the result of entangled polymers of mutant Z alpha1-antitrypsin.
15 e expression, including HNF4, HNF1alpha, and alpha1-antitrypsin.
16 formation of heteropolymers between S and Z alpha1-antitrypsin.
17 d for the formation of a stable complex with alpha1-antitrypsin.
18 important component of the function of human alpha1-antitrypsin.
19 es of the APPs transferrin, haptoglobin, and alpha1-antitrypsin.
20 ut compromising the inhibitory activity of Z alpha1-antitrypsin.
21 a loss of anti-inflammatory signalling by M alpha1-antitrypsin.
22 two GVHD severity markers, calprotectin and alpha1-antitrypsin.
23 , and this could be inhibited by addition of alpha1-antitrypsin.
24 nation and proteasomal degradation of mutant alpha1-antitrypsin.
25 emphysema caused by mutations in the serpin 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
38 C and alcoholism developed large numbers of alpha1-antitrypsin (AAT) globules within hepatocytes of
42 istration of the serine proteinase inhibitor alpha1-antitrypsin (AAT) prevents type 1 diabetes develo
43 uble/insoluble distribution of two misfolded alpha1-antitrypsin (AAT) variants responsible for AAT de
45 s were selected from the proteomic analysis, alpha1-antitrypsin (AAT), hemopexin (HX), and gelsolin (
48 on, kidney lysosomal enzyme elevation, serum alpha1-antitrypsin activity deficiency and abnormal otol
52 e ER of liver cells in the classical form of alpha1 antitrypsin (alpha1-AT) deficiency is associated
53 receptor initially identified for binding of alpha1-antitrypsin (alpha1-AT) and other serine protease
57 t ER degradation of mutant secretory protein alpha1-antitrypsin (alpha1-AT) Z, the mutant protein ass
59 d levels of IL-8, protein, LDH, fibronectin, alpha1-antitrypsin (alpha1-AT), complement fragment 3a (
60 e inhibitor of metalloproteinase-1 (TIMP-1), alpha1-antitrypsin (alpha1-AT), protease nexin II (PN-II
62 ription of three HNF-4alpha sensitive genes, alpha1-antitrypsin (alpha1-AT), transthyretin (TTR), and
65 are mainly expressed in the liver, including alpha1-antitrypsin, alpha1-antichymotrypsin, alpha-fetal
68 last somatic cell hybrids, extinction of rat alpha1-antitrypsin (alpha1AT) gene expression is accompa
70 s control region (LCR) upstream of the human alpha1-antitrypsin (alpha1AT) gene that is required for
74 (Glu342Lys) in the serine protease inhibitor alpha1-antitrypsin (alpha1AT), which is found in more th
75 in, heparin cofactor II (HCII)-thrombin, and alpha1-antitrypsin (alpha1AT)-trypsin bound to purified
76 serpin family: protein C inhibitor (PCI) and alpha1-antitrypsin (alpha1AT); however, both exhibit poo
82 production could be recovered by addition of alpha1-antitrypsin, an endogenous inhibitor of serine pr
83 reported here of a mixed heterozygote for Z alpha1-antitrypsin and another conformationally unstable
84 homologous in sequence and function to human alpha1-antitrypsin and are encoded by a highly conserved
86 diazole) was used to label peroxide-modified alpha1-antitrypsin and demonstrate that the Cys-232 in v
89 physical association between unfolded human alpha1-antitrypsin and UDP-glucose:glycoprotein glucosyl
90 se had measurements of fecal lactoferrin and alpha1-antitrypsin and underwent pouch endoscopy with bi
91 correlate well with immunological levels of alpha1-antitrypsin and, thus, may prove useful for asses
92 f two serine protease inhibitors [Serpina1a (alpha1-antitrypsin) and Elafin] was dysregulated in Fbln
93 enteropathy (calprotectin, myeloperoxidase, alpha1-antitrypsin) and the prevalence of bacterial but
94 sponse genes such as SERPINA1, which encodes alpha1 antitrypsin, and FOXP4, an inhibitor of mucus pro
95 itors of metalloproteinase 2, -3, and -4 and alpha1-antitrypsin, and fibrosis was associated with inc
96 R1, TNFR2, Bid), optimal IL-13 inhibition of alpha1-antitrypsin, and IL-13-induction of and activatio
97 molecules, the solubility of mutant forms of alpha1-antitrypsin, and interactions with newly synthesi
98 es) is the target for protease inhibition by alpha1-antitrypsin, and its unopposed release destroys t
99 oembryonic antigen, retinol binding protein, alpha1-antitrypsin, and squamous cell carcinoma antigen-
100 termined levels of carcinoembryonic antigen, alpha1-antitrypsin, and squamous cell carcinoma antigen.
103 tracellular serpins such as antithrombin and alpha1-antitrypsin are the quintessential regulators of
104 ave assessed a surface hydrophobic cavity in alpha1-antitrypsin as a potential target for rational dr
105 ts identifying cathepsin C, cathepsin Z, and alpha1-antitrypsin as additional potential cargoes for L
109 hial epithelial cells with purified plasma M alpha1-antitrypsin attenuates this inflammatory response
110 echanism due to accumulation of the mutant Z alpha1-antitrypsin (ATZ) and is a key example of an dise
111 C-terminal end of the serpin (e.g. FVFLM in alpha1-antitrypsin) binds to the serpin-enzyme complex r
112 (native), thrombin-antithrombin, or elastase-alpha1-antitrypsin, but not by free HCII or thrombin, wh
113 mutation reduces concentrations in serum of alpha1 antitrypsin by retaining polymerised molecules wi
115 reduced the intracellular accumulation of Z alpha1-antitrypsin by 70% in a cell model of disease.
116 biomarkers, followed by IL-2 receptor alpha, alpha1-antitrypsin, C-reactive protein, YKL-40, cellular
121 ent here the 2.6 A structure of a polymer of alpha1-antitrypsin cleaved six residues N-terminal to th
122 philia and plasma concentrations of elastase-alpha1-antitrypsin complexes and lactoferrin), but did n
123 f interleukin-6, interleukin-8, and elastase-alpha1-antitrypsin complexes compared with presurgery le
124 f interleukin-8, interleukin-6, and elastase-alpha1-antitrypsin complexes were elevated compared with
126 hil degranulation (plasma levels of elastase-alpha1-antitrypsin complexes, P < .05) and modestly redu
127 ls of interleukin-6, interleukin-8, elastase-alpha1-antitrypsin complexes, thrombin-antithrombin comp
128 Treatment with the serine protease inhibitor alpha1-antitrypsin decreased serum levels of HS, leading
130 y centres in 13 countries if they had severe alpha1 antitrypsin deficiency (serum concentration <11 m
131 sensitive measure of disease progression in alpha1 antitrypsin deficiency emphysema than spirometry
132 inhibitor (A1PI) augmentation treatment for alpha1 antitrypsin deficiency has not been substantiated
133 mphysema progression in patients with severe alpha1 antitrypsin deficiency in a randomised controlled
142 verity and distribution in 119 subjects with alpha1-antitrypsin deficiency (PiZ phenotype) and groupe
147 ng of genetic and nongenetic modifiers in ZZ alpha1-antitrypsin deficiency and other disorders of pro
148 the end-stage liver disease associated with alpha1-antitrypsin deficiency and underscore the contrib
149 he most frequent mutation that causes severe alpha1-antitrypsin deficiency arises in the SERPINA 1 ge
151 ents with CF, primary ciliary dyskinesia, or alpha1-antitrypsin deficiency exhibited 3-fold higher mu
158 netic disorders, such as cystic fibrosis and alpha1-antitrypsin deficiency, and for other diseases, i
159 with either alcohol-related liver disease or alpha1-antitrypsin deficiency, and only one of the healt
160 Less common causes include hemochromatosis, alpha1-antitrypsin deficiency, autoimmune hepatitis, and
161 blished pulmonary mechanics in patients with alpha1-antitrypsin deficiency, chronic obstructive pulmo
162 ess of augmentation therapy (Aug) for severe alpha1-antitrypsin deficiency, comparing strategies of:
163 abolic conditions studied in further detail (alpha1-antitrypsin deficiency, familial hypercholesterol
174 tients with alcohol-related liver disease or alpha1-antitrypsin-deficiency liver disease, and only on
175 ther hereditary iron overload disorders, and alpha1-antitrypsin disease-are the focus of this review.
177 equence corresponding to residues 359-374 of alpha1-antitrypsin, enhances gene expression from DNA na
179 rects calnexin in the selection of misfolded alpha1-antitrypsin for degradation by the proteasome.
180 eatments for emphysema, infusion of purified alpha1 antitrypsin from pooled human plasma represents a
181 ccharides abrogates the release of misfolded alpha1-antitrypsin from calnexin prior to proteasomal de
184 of an 8-kb DNA segment upstream of the human alpha1-antitrypsin gene yields a mutant serpin allele th
186 A transgenic mouse line expressing the human alpha1-antitrypsin (hA1AT) gene was developed in an FVB/
187 " hepatocytes from mice transgenic for human alpha1-antitrypsin (hA1AT) were transplanted by intraspl
191 gents, monotherapy with clinical-grade human alpha1-antitrypsin (hAAT), the major serum serine-protea
192 ecreased the persistence of transgene (human alpha1-antitrypsin [hAAT]) expression that was associate
193 Ad) vector-encoded proteins, including human alpha1-antitrypsin (huAAT), mouse erythropoietin (EPO),
194 TGN, where it stimulates the sialylation of alpha1-antitrypsin (i.e. one of the identified secretory
195 denoassociated virus vector expressing human alpha1-antitrypsin in murine liver progenitor cells.
196 re derived, such as aggregation of misfolded alpha1-antitrypsin in the endoplasmic reticulum, deficie
197 e accumulation of the misfolded Z variant of alpha1-antitrypsin in the hepatocyte endoplasmic reticul
201 findings have indicated that a deficiency in alpha1-antitrypsin is associated with increased risk of
202 ellular portion of the pIgR, linked to human alpha1-antitrypsin is effectively ferried across human t
204 disease, whereas low levels of circulating Z alpha1-antitrypsin lead to emphysema by loss of inhibiti
206 nt human MARs, from the apolipoprotein B and alpha1-antitrypsin loci, insulated white transgene expre
207 cal production of polymers by mutant S and Z alpha1-antitrypsin may have also provided protection aga
208 in addition to its antielastolytic effects, alpha1-antitrypsin may have broader biological effects i
209 n-originated cells expressing liver-specific alpha1-antitrypsin messenger RNA, albumin and hepatocyte
210 lt in a conformational transition within the alpha1-antitrypsin molecule and the formation of polymer
211 se protective, proinflammatory properties of alpha1-antitrypsin mutants have become detrimental to ca
212 ts were effective at ratios of compound to Z alpha1-antitrypsin of 2.5:1 and reduced the intracellula
213 ex, did not alter the inactivation of APC by alpha1-antitrypsin or protein C inhibitor, and did not i
214 helium protease is not highly susceptible to alpha1-antitrypsin or secretory leukocyte protease inhib
215 The mutation in the Z deficiency variant of alpha1-antitrypsin perturbs the structure of the protein
216 es deleted and containing the complete human alpha1-antitrypsin (PI) locus, we observed tissue-specif
217 a framework for understanding the uncleaved alpha1-antitrypsin polymer and fibrillar and amyloid dep
218 allow us to propose a kinetic mechanism for alpha1-antitrypsin polymer formation that involves the g
219 the generation of an mAb (4B12) that blocked alpha1-antitrypsin polymerization in vitro at a 1:1 mola
220 iophysical techniques have demonstrated that alpha1-antitrypsin polymerization is a two-stage process
222 e the utility of the protein-based inhibitor alpha1-antitrypsin Portland (alpha1-PDX) as an antipatho
223 pressed a potent protein inhibitor of furin, alpha1-antitrypsin Portland (alpha1-PDX) in early Xenopu
224 substrate decanoyl-RVKR-chloromethylketone, alpha1-antitrypsin Portland and by its own propeptide.
225 study, we show that inducible expression of alpha1-antitrypsin Portland, a furin inhibitor, inhibits
227 ific promoter (murine albumin enhancer/human alpha1-antitrypsin promoter) further enhanced transgene
229 lation of the TGF-beta signaling pathway and alpha1-antitrypsin protein (a serine protease inhibitor)
230 e disease, inefficient secretion of a mutant alpha1-antitrypsin protein (AAT-Z) results in its accumu
231 of several tested in yielding expression of alpha1-antitrypsin protein from a retroviral vector in h
233 ummary, this work provides new insights into alpha1-antitrypsin reactivity in oxidizing environments
234 brane protein, or the soluble PiZ variant of alpha1-antitrypsin, reduced levels of general COPII vesi
235 ion of structural alveolar cell apoptosis by alpha1-antitrypsin represents a novel protective mechani
236 oyed to investigate quality control of human alpha1-antitrypsin secretion from stably transfected mou
238 -fold; apolipoprotein A-1 [APOA1], 3.2-fold; alpha1-antitrypsin [SERPINA1], 2.5-fold; and complement
239 By injection of plasmid DNA encoding human alpha1-antitrypsin, significant concentrations of hAAT w
240 s containing an immobile matrix of polymeric alpha1-antitrypsin, small ER resident proteins can diffu
241 Ab technology to identify interactors with Z alpha1-antitrypsin that comply with both requirements.
242 tify a peptide corresponding to a portion of alpha1-antitrypsin that potently inhibits entry of HIV-1
243 intrabody also increased the secretion of Z alpha1-antitrypsin that retained inhibitory activity aga
244 but did increase the levels of mRNA encoding alpha1-antitrypsin, tissue inhibitor of metalloproteinas
245 tissue and the high risk of patients lacking alpha1-antitrypsin to develop emphysema, much interest h
246 Using cytomegalovirus (CMV)-driven human alpha1-antitrypsin, transgene expression was immunolocal
247 rved multiply charged states at m/z 72,160 ([alpha1-antitrypsin + trypsin + H](+)) and 86,585 ([IgG +
248 atrix for the detection of several proteins (alpha1-antitrypsin, trypsin, IgG, protein G) and their c
249 86,585 ([IgG + protein G + 2H](2+)) for the alpha1-antitrypsin-trypsin and IgG-protein G complexes,
250 detection of weak protein complexes, such as alpha1-antitrypsin-trypsin and IgG-protein G complexes,
251 Plasma concentrations of C-reactive protein, alpha1-antitrypsin, tumor necrosis factor alpha (TNF-alp
254 NTS, AND MAIN RESULTS: Transduction of human alpha1-antitrypsin via replication-deficient adeno-assoc
255 acellular fate of terminally misfolded human alpha1-antitrypsin was examined in hepatoma cells to ide
257 errant form of the hepatic secretory protein alpha1-antitrypsin was stably expressed in a human embry
258 addition, transgene expression (serum human alpha1-antitrypsin) was sustained for the length of the
259 ch corresponds to Met(358), the P(1) site of alpha1-antitrypsin, was the inhibitory site for elastase
260 noassociated virus 1-vector-expressing human alpha1 antitrypsin were transplanted into the liver of m
261 Escherichia coli beta-galactosidase or human alpha1-antitrypsin were prepared and expressed the repor
262 reactive loop and shutter domain mutants of alpha1-antitrypsin were used to demonstrate the close as
263 tive-to-latent transition of another serpin, alpha1-antitrypsin, which does not readily go latent.
264 commonest pathogenic gene mutation yields Z-alpha1-antitrypsin, which has a propensity to self-assoc
266 2E1 (CYP2E1) by measuring the expression of alpha1-antitrypsin, which is controlled by these promote
267 for the Z variant, but even more common is S alpha1-antitrypsin, which is found in up to 28% of south
268 serpins (antithrombin, protease nexin-1 and alpha1-antitrypsin with a P1 arginine) were 2 x 10(3) to
269 t of soluble secretory proteins (albumin and alpha1-antitrypsin) with that of supramolecular cargoes
272 tracellular accumulation of misfolded mutant alpha1-antitrypsin Z (ATZ) in hepatocytes causes hepatic
273 characterized by accumulation of the mutant alpha1-antitrypsin Z (ATZ) variant inside cells, causing
275 modifiers affecting the accumulation of the alpha1-antitrypsin Z mutant (ATZ) in a Caenorhabditis el
276 transgenic for the common misfolded variant alpha1-antitrypsin Z, is a model of respiratory epitheli
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