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Review

The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals

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Pages 123-134 | Published online: 26 Mar 2018

References

  • KhanMSSinghPAzharASerpin inhibition mechanism: a delicate balance between native metastable state and polymerizationJ Amino Acids2011201160679722312466
  • CoxDWMarkovicVDTeshimaIEGenes for immunoglobulin heavy chains and for alpha 1-antitrypsin are localized to specific regions of chromosome 14qNature198229758654284306804874
  • PerlmutterDHColeFSKilbridgePRossingTHColtenHRExpression of the alpha 1-proteinase inhibitor gene in human monocytes and macrophagesProceedings of the National Academy of Sciences1985823795799
  • du BoisRMBernaudinJFPaakkoPHuman neutrophils express the alpha 1-antitrypsin gene and produce alpha 1-antitrypsinBlood19917712272427302043769
  • PerlmutterDHKayRMColeFSRossingTHVan ThielDColtenHRThe cellular defect in alpha 1-proteinase inhibitor (alpha 1-PI) deficiency is expressed in human monocytes and in Xenopus oocytes injected with human liver mRNAProc Natl Acad Sci U S A19858220691869213876562
  • MolmentiEPPerlmutterDHRubinDCCell-specific expression of alpha 1-antitrypsin in human intestinal epitheliumJ Clin Invest1993924202220348408656
  • BoscoDMedaPMorelPExpression and secretion of alpha1-proteinase inhibitor are regulated by proinflammatory cytokines in human pancreatic islet cellsDiabetologia20054881523153316001235
  • ChenXLZhouLYangJShenFKZhaoSPWangYLHepatocellular carcinoma-associated protein markers investigated by MALDI-TOF MSMol Med Rep20103458959621472284
  • LewisECExpanding the clinical indications for α(1)-antitrypsin therapyMol Med20121895797022634722
  • JonesEAVergallaJSteerCJBradley-MoorePRVierlingJMMetabolism of intact and desialylated alpha 1-antitrypsinClin Sci Mol Med1978552139148307998
  • PerlmutterDHAlpha-1-antitrypsin deficiency: diagnosis and treatmentClin Liver Dis200484839859viiiix15464658
  • JanciauskieneSMBalsRKoczullaRVogelmeierCKöhnleinTWelteTThe discovery of α1-antitrypsin and its role in health and diseaseRespir Med201110581129113921367592
  • GreeneCMMcElvaneyNGProteases and antiproteases in chronic neutrophilic lung disease – relevance to drug discoveryBr J Pharmacol200915841048105819845686
  • ElliottPRPeiXYDaffornTRLomasDATopography of a 2.0 A structure of alpha1-antitrypsin reveals targets for rational drug design to prevent conformational diseaseProtein Sci2000971274128110933492
  • KolarichDTurecekPLWeberABiochemical, molecular characterization, and glycoproteomic analyses of alpha(1)-proteinase inhibitor products used for replacement therapyTransfusion200646111959197717076852
  • McCarthyCDunleaDMSaldovaRGlycosylation repurposes alpha-1 antitrypsin for resolution of community-acquired-pneumoniaAm J Respir Crit Care Med Epub20171116
  • PaceCNConformational stability of globular proteinsTrends Biochem Sci199015114172107612
  • PerlmutterDHJoslinGNelsonPSchasteenCAdamsSPFallonRJEndocytosis and degradation of alpha 1-antitrypsin-protease complexes is mediated by the serpin-enzyme complex (SEC) receptorJ Biol Chem19902652816713167162211587
  • Chorostowska-WynimkoJStruniawskiRPopławskaBBorszewska-KornackaMThe incidence of alpha-1-antitrypsin (A1AT) deficiency alleles in population of Central Poland–preliminary results from newborn screeningPneumonol Alergol Pol2012805450453 Polish [with English abstract]22926906
  • PopławskaBJanciauskieneSChorostowska-WynimkoJGenetic variants of alpha-1 antitrypsin: classification and clinical implicationsPneumonol Alergol Pol20138114554 Polish [with English abstract]23258471
  • SesboüéRMartinJPAlpha-1-antitrypsin (PI) polymorphism in France, with special regard to the PI*Z alleleHum Hered19914153403461778610
  • MassiGFabianoARagusaDAlpha 1-antitrypsin phenotypes and pi m subtypes in ItalyBull Eur Physiopathol Respir198016Suppl3013066971674
  • CarrollTPO’ConnorCAFloydOThe prevalence of alpha-1 antitrypsin deficiency in IrelandRespir Res2011129121752289
  • CrystalRGAlpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapyJ Clin Invest1990855134313522185272
  • DeMeoDLSilvermanEKAlpha1-antitrypsin deficiency. 2: genetic aspects of alpha(1)-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema riskThorax200459325926414985567
  • CurielDChytilACourtneyMCrystalRGSerum alpha 1-antitrypsin deficiency associated with the common S-type (Glu264—Val) mutation results from intracellular degradation of alpha 1-antitrypsin prior to secretionJ Biol Chem19892641810477104862567291
  • LomasDAEvansDLFinchJTCarrellRWThe mechanism of Z alpha 1-antitrypsin accumulation in the liverNature199235763796056071608473
  • BrantlyMNukiwaTCrystalRGMolecular basis of alpha-1-antitrypsin deficiencyAm J Med1988846A13313289385
  • MolloyKHershCPMorrisVBClarification of the risk of chronic obstructive pulmonary disease in α1-antitrypsin deficiency PiMZ heterozygotesAm J Respir Crit Care Med2014189441942724428606
  • KellyEGreeneCMCarrollTPMcElvaneyNGO’NeillSJAlpha-1 antitrypsin deficiencyRespir Med2010104676377220303723
  • JanoffAElastase in tissue injuryAnnu Rev Med1985362072163888051
  • CoxDWLevisonHEmphysema of early onset associated with a complete deficiency of alpha-1-antitrypsin (null homozygotes)Am Rev Respir Dis198813723713753257661
  • NukiwaTTakahashiHBrantlyMCourtneyMCrystalRGalpha 1-Antitrypsin nullGranite Falls, a nonexpressing alpha 1-antitrypsin gene associated with a frameshift to stop mutation in a coding exonJ Biol Chem19872622511999120043040726
  • ParrDGStoelBCStolkJStockleyRAPattern of emphysema distribution in alpha1-antitrypsin deficiency influences lung function impairmentAm J Respir Crit Care Med2004170111172117815306534
  • TomashefskiJFJrCrystalRGWiedemannHPMaschaEStollerJKAlpha 1-Antitrypsin Deficiency Registry Study GroupThe bronchopulmonary pathology of alpha-1 antitrypsin (AAT) deficiency: findings of the Death Review Committee of the national registry for individuals with Severe Deficiency of Alpha-1 AntitrypsinHum Pathol200435121452146115619203
  • KorkmazBAttucciSEpinetteCMeasurement of neutrophil elastase, proteinase 3, and cathepsin G activities using intramolecularly quenched fluorogenic substratesMethods Mol Biol201284412513822262439
  • TaggartCCervantes-LaureanDKimGOxidation of either methionine 351 or methionine 358 in alpha 1-antitrypsin causes loss of anti-neutrophil elastase activityJ Biol Chem200027535272582726510867014
  • McCarthyCSaldovaRWormaldMRRuddPMMcElvaneyNGReevesEPThe role and importance of glycosylation of acute phase proteins with focus on alpha-1 antitrypsin in acute and chronic inflammatory conditionsJ Proteome Res20141373131314324892502
  • KasturiLEshlemanJRWunnerWHShakin-EshlemanSHThe hydroxy amino acid in an Asn-X-Ser/Thr sequon can influence N-linked core glycosylation efficiency and the level of expression of a cell surface glycoproteinJ Biol Chem19952702414756147617782341
  • BetsuyakuTNishimuraMTakeyabuKTaninoMMiyamotoKKawakamiYDecline in FEV(1) in community-based older volunteers with higher levels of neutrophil elastase in bronchoalveolar lavage fluidRespiration200067326126710867593
  • SilbersteinSGilmoreRBiochemistry, molecular biology, and genetics of the oligosaccharyltransferaseFASEB J19961088498588666161
  • SchönbergMReibetanzURathmannSLessigJMaintenance of α(1)-antitrypsin activity by means of co-application of hypochlorous acid-scavengers in vitro and in the supernatant of polymorphonuclear leukocytes: as a basis for a new drug delivery approachBiomatter201221243623507783
  • SegelmarkMElzoukiANWieslanderJErikssonSThe PiZ gene of alpha 1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitisKidney Int19954838448507474674
  • PittelkowMRSmithKCSuWPAlpha-1-antitrypsin deficiency and panniculitis. Perspectives on disease relationship and replacement therapyAm J Med1988846A80863260076
  • HubbardRCFellsGGadekJPacholokSHumesJCrystalRGNeutrophil accumulation in the lung in alpha 1-antitrypsin deficiency. Spontaneous release of leukotriene B4 by alveolar macrophagesJ Clin Invest19918838918971653278
  • GalliSJBorregaardNWynnTAPhenotypic and functional plasticity of cells of innate immunity: macrophages, mast cells and neutrophilsNat Immunol201112111035104422012443
  • HaraokaMHangLFrendéusBNeutrophil recruitment and resistance to urinary tract infectionJ Infect Dis199918041220122910479151
  • SummersCRankinSMCondliffeAMSinghNPetersAMChilversERNeutrophil kinetics in health and diseaseTrends Immunol201031831832420620114
  • OwenCACampbellMABoukedesSSCampbellEJCytokines regulate membrane-bound leukocyte elastase on neutrophils: a novel mechanism for effector activityAm J Physiol19972723 Pt 1L385L3939124593
  • WeissSJRegianiSNeutrophils degrade subendothelial matrices in the presence of alpha-1-proteinase inhibitor. Cooperative use of lysosomal proteinases and oxygen metabolitesJ Clin Invest1984735129713036325501
  • BelaaouajAMcCarthyRBaumannMMice lacking neutrophil elastase reveal impaired host defense against gram negative bacterial sepsisNat Med1998456156189585238
  • GuyotNWartelleJMalleretLUnopposed cathepsin G, neutrophil elastase, and proteinase 3 cause severe lung damage and emphysemaAm J Pathol201418482197221024929239
  • SmedlyLATonnesenMGSandhausRANeutrophil-mediated injury to endothelial cells. Enhancement by endotoxin and essential role of neutrophil elastaseJ Clin Invest1986774123312433485659
  • TravisJStructure, function, and control of neutrophil proteinasesAm J Med1988846A3742
  • LundgrenJDRievesRDMullolJLogunCShelhamerJHThe effect of neutrophil proteinase enzymes on the release of mucus from feline and human airway culturesRespir Med19948875115187972975
  • KimKCWasanoKNilesRMSchusterJEStonePJBrodyJSHuman neutrophil elastase releases cell surface mucins from primary cultures of hamster tracheal epithelial cellsProc Natl Acad Sci U S A19878424930493083480544
  • GaggarALiYWeathingtonNMatrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patientsAm J Physiol Lung Cell Mol Physiol20072931L96L10417384080
  • ZhuYKLiuXDSköldCMSynergistic neutrophil elastase-cytokine interaction degrades collagen in three-dimensional cultureAm J Physiol Lung Cell Mol Physiol20012814L868L87811557590
  • GuyotNButlerMWMcNallyPElafin, an elastase-specific inhibitor, is cleaved by its cognate enzyme neutrophil elastase in sputum from individuals with cystic fibrosisJ Biol Chem200828347323773238518799464
  • WeldonSMcNallyPMcElvaneyNGDecreased levels of secretory leucoprotease inhibitor in the Pseudomonas-infected cystic fibrosis lung are due to neutrophil elastase degradationJ Immunol2009183128148815620007580
  • BergerMSorensenRUTosiMFDearbornDGDöringGComplement receptor expression on neutrophils at an inflammatory site, the Pseudomonas-infected lung in cystic fibrosisJ Clin Invest1989844130213132507578
  • TosiMFZakemHBergerMNeutrophil elastase cleaves C3bi on opsonized pseudomonas as well as CR1 on neutrophils to create a functionally important opsonin receptor mismatchJ Clin Invest19908613003082164045
  • HartlDLatzinPHordijkPCleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung diseaseNat Med200713121423143018059279
  • Vega-CarrascalIBerginDAMcElvaneyOJGalectin-9 signaling through TIM-3 is involved in neutrophil-mediated Gram-negative bacterial killing: an effect abrogated within the cystic fibrosis lungJ Immunol201419252418243124477913
  • Vega-CarrascalIReevesEPNikiTDysregulation of TIM-3-galectin-9 pathway in the cystic fibrosis airwaysJ Immunol201118652897290921263071
  • FickRBJrNaegelGPSquierSUWoodREGeeJBReynoldsHYProteins of the cystic fibrosis respiratory tract. Fragmented immunoglobulin G opsonic antibody causing defective opsonophagocytosisJ Clin Invest19847412362486429195
  • ReevesEPWilliamsonMByrneBIL-8 dictates glycosaminoglycan binding and stability of IL-18 in cystic fibrosisJ Immunol201018431642165220026745
  • CarrellRWJeppssonJOLaurellCBStructure and variation of human alpha 1-antitrypsinNature198229858723293347045697
  • Vercaigne-MarkoDDavrilMLaineAHayemAInteraction of human alpha1-proteinase inhibitor with human leukocyte cathepsin GBiol Chem Hoppe Seyler198536676556613876103
  • DurantonJBiethJGInhibition of proteinase 3 by [alpha]1-antitrypsin in vitro predicts very fast inhibition in vivoAm J Respir Cell Mol Biol2003291576112600819
  • YingQLSimonSRElastolysis by proteinase 3 and its inhibition by alpha(1)-proteinase inhibitor: a mechanism for the incomplete inhibition of ongoing elastolysisAm J Respir Cell Mol Biol200226335636111867344
  • FalkRJTerrellRSCharlesLAJennetteJCAnti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitroProc Natl Acad Sci U S A19908711411541192161532
  • RouhaniFPaoneGSmithNKKreinPBarnesPBrantlyMLLung neutrophil burden correlates with increased pro-inflammatory cytokines and decreased lung function in individuals with alpha(1)-antitrypsin deficiencyChest20001175 Suppl 1250S251S10843938
  • ElliottPRBiltonDLomasDALung polymers in Z alpha1-antitrypsin deficiency-related emphysemaAm J Respir Cell Mol Biol19981856706749569237
  • MahadevaRAtkinsonCLiZPolymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivoAm J Pathol2005166237738615681822
  • MulgrewATTaggartCCLawlessMWZ alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractantChest200412551952195715136414
  • ParmarJSMahadevaRReedBJPolymers of alpha(1)-anti-trypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysemaAm J Respir Cell Mol Biol200226672373012034572
  • LomasDACarrellRWSerpinopathies and the conformational dementiasNat Rev Genet200231075976812360234
  • WuYWhitmanIMolmentiEMooreKHippenmeyerPPerlmutterDHA lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiencyProc Natl Acad Sci U S A19949119901490188090762
  • HurleyKLaceyNO’DwyerCAAlpha-1 antitrypsin augmentation therapy corrects accelerated neutrophil apoptosis in deficient individualsJ Immunol201419383978399125217166
  • BlancoILipskerDLaraBJanciauskieneSNeutrophilic panniculitis associated with alpha-1-antitrypsin deficiency: an updateBr J Dermatol2016174475376226595240
  • GrossBGrebeMWenckerMStollerJKBjurstenLMJanciauskieneSNew findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapyDermatology2009218437037519218787
  • KunkelSLStandifordTKasaharaKStrieterRMInterleukin-8 (IL-8): the major neutrophil chemotactic factor in the lungExp Lung Res199117117232013270
  • MalerbaMRicciardoloFRadaeliANeutrophilic inflammation and IL-8 levels in induced sputum of alpha-1-antitrypsin PiMZ subjectsThorax200661212913316284217
  • DetmersPALoSKOlsen-EgbertEWalzABaggioliniMCohnZANeutrophil-activating protein 1/interleukin 8 stimulates the binding activity of the leukocyte adhesion receptor CD11b/CD18 on human neutrophilsJ Exp Med19901714115511621969919
  • ZeilhoferHUSchorrWRole of interleukin-8 in neutrophil signalingCurr Opin Hematol20007317818210786656
  • DevaneyJMGreeneCMTaggartCCCarrollTPO’NeillSJMcElvaneyNGNeutrophil elastase up-regulates interleukin-8 via toll-like receptor 4FEBS Lett20035441–312913212782302
  • OstrowskaESokolovaEReiserGPAR-2 activation and LPS synergistically enhance inflammatory signaling in airway epithelial cells by raising PAR expression level and interleukin-8 releaseAm J Physiol Lung Cell Mol Physiol20072935L1208L121817766588
  • RamachandranRHollenbergMDProteinases and signalling: pathophysiological and therapeutic implications via PARs and moreBr J Pharmacol2008153Suppl 1S263S28218059329
  • BerginDAReevesEPMeleadyPα-1 antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8J Clin Invest2010120124236425021060150
  • JonigkDAl-OmariMMaegelLAnti-inflammatory and immunomodulatory properties of α1-antitrypsin without inhibition of elastaseProc Natl Acad Sci U S A201311037150071501223975926
  • Ford-HutchinsonAWBrayMADoigMVShipleyMESmithMJLeukotriene B, a potent chemokinetic and aggregating substance released from polymorphonuclear leukocytesNature198028657702642656250050
  • HooverRLKarnovskyMJAustenKFCoreyEJLewisRALeukotriene B4 action on endothelium mediates augmented neutrophil/endothelial adhesionProc Natl Acad Sci U S A1984817219121936326110
  • O’DwyerCAO’BrienMEWormaldMRThe BLT1 inhibitory function of alpha-1 antitrypsin augmentation therapy disrupts leukotriene B4 neutrophil signalingJ Immunol201519583628364126371243
  • BerginDAReevesEPHurleyKThe circulating proteinase inhibitor α-1 antitrypsin regulates neutrophil degranulation and autoimmunitySci Transl Med20146217217ra1
  • BrandtEPetersenFFladHDRecombinant tumor necrosis factor-alpha potentiates neutrophil degranulation in response to host defense cytokines neutrophil-activating peptide 2 and IL-8 by modulating intracellular cyclic AMP levelsJ Immunol19921494135613641323612
  • MócsaiAJakusZVántusTBertonGLowellCALigetiEKinase pathways in chemoattractant-induced degranulation of neutrophils: the role of p38 mitogen-activated protein kinase activated by Src family kinasesJ Immunol200016484321433110754332
  • Van MolleWLibertCFiersWBrouckaertPAlpha 1-acid glycoprotein and alpha 1-antitrypsin inhibit TNF-induced but not anti-Fas-induced apoptosis of hepatocytes in miceJ Immunol19971597355535649317155
  • ZhangBLuYCampbell-ThompsonMAlpha1-antitrypsin protects beta-cells from apoptosisDiabetes20075651316132317360983
  • GraziadeiIKaserbacherRBraunsteinerHVogelWThe hepatic acute-phase proteins alpha 1-antitrypsin and alpha 2-macroglobulin inhibit binding of transferrin to its receptorBiochem J1993290Pt 11091137679893
  • SchmidtEWRascheBUlmerWTReplacement therapy for alpha-1-protease inhibitor deficiency in PiZ subjects with chronic obstructive lung diseaseAm J Med1988846A63693289388
  • WewersMDCasolaroMASellersSEReplacement therapy for alpha 1-antitrypsin deficiency associated with emphysemaN Engl J Med198731617105510623494198
  • ChapmanKRBurdonJGPiitulainenERAPID Trial Study GroupIntravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trialLancet2015386999136036826026936
  • McElvaneyNGBurdonJHolmesMRAPID Extension Trial GroupLong-term efficacy and safety of α1 proteinase inhibitor treatment for emphysema caused by severe α1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE)Lancet Respir Med201751516027916480
  • LiebermanJAugmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting dataChest200011851480148511083705
  • MolloyMO’ConnorCFeeLCarrollTPMcElvaneyNGReal life treatment benefit of intravenous augmentation therapy for severe alpha-1 antitrypsinIr J Med Sci20161859S439S508
  • SmithKCPittelkowMRSuWPPanniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literatureArch Dermatol198712312165516613318708
  • FranciosiANMcCarthyCCarrollTPMcElvaneyNGUnusual acute sequelae of α1-antitrypsin deficiency: a myriad of symptoms with one common cureChest20151485e136e13826527439
  • DowdSKRodgersGCCallenJPEffective treatment with alpha 1-protease inhibitor of chronic cutaneous vasculitis associated with alpha 1-antitrypsin deficiencyJ Am Acad Dermatol1995335 Pt 29139167593810
  • GrieseMLatzinPKapplerMAlpha1-antitrypsin inhalation reduces airway inflammation in cystic fibrosis patientsEur Respir J200729224025017050563
  • PetracheIFijalkowskaIMedlerTRAlpha-1 antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosisAm J Pathol200616941155116617003475
  • LewisECShapiroLBowersOJDinarelloCAAlpha1-antitrypsin monotherapy prolongs islet allograft survival in miceProc Natl Acad Sci U S A200510234121531215816093309
  • MolanoRDPileggiASongSProlonged islet allograft survival by alpha-1 antitrypsin: the role of humoral immunityTransplant Proc200840245545618374099
  • PileggiAMolanoRDSongSAlpha-1 antitrypsin treatment of spontaneously diabetic nonobese diabetic mice receiving islet allograftsTransplant Proc200840245745818374100
  • KalisMKumarRJanciauskieneSSalehiACilioCMα 1-Antitrypsin enhances insulin secretion and prevents cytokine-mediated apoptosis in pancreatic β-cellsIslets20102318518921099312
  • BerginDAHurleyKMcElvaneyNGReevesEPAlpha-1 antitrypsin: a potent anti-inflammatory and potential novel therapeutic agentArch Immunol Ther Exp (Warsz)2012602819722349104
  • McElvaneyNGAlpha-1 antitrypsin therapy in cystic fibrosis and the lung disease associated with alpha-1 antitrypsin deficiencyAnn Am Thorac Soc201613Suppl 2S191S19627115956
  • McElvaneyNGHubbardRCBirrerPAerosol alpha 1-antitrypsin treatment for cystic fibrosisLancet199133787383923941671425
  • MartinSLDowneyDBiltonDKeoganMTEdgarJElbornJSRecombinant AAT CF Study TeamSafety and efficacy of recombinant alpha(1)-antitrypsin therapy in cystic fibrosisPediatr Pulmonol200641217718316372352
  • CantinAMBerthiaumeYCloutierDMartelMProlastin aerosol therapy and sputum taurine in cystic fibrosisClin Invest Med200629420120716986483
  • VerledenGMRaghuGMeyerKCGlanvilleARCorrisPA new classification system for chronic lung allograft dysfunctionJ Heart Lung Transplant201433212713324374027
  • LiorYGeyraALewisECTherapeutic compositions and uses of alpha1-antitrypsin: a patent review (2012–2015)Expert Opin Ther Pat201626558158926966859
  • DaemenMAHeemskerkVHvan’t VeerCFunctional protection by acute phase proteins alpha(1)-acid glycoprotein and alpha(1)-antitrypsin against ischemia/reperfusion injury by preventing apoptosis and inflammationCirculation2000102121420142610993862
  • KanerZOchayonDEShahafGAcute phase protein α1-antitrypsin reduces the bacterial burden in mice by selective modulation of innate cell responsesJ Infect Dis201521191489149825389308
  • KoulmandaMBhasinMHoffmanLCurative and beta cell regenerative effects of alpha1-antitrypsin treatment in autoimmune diabetic NOD miceProc Natl Acad Sci U S A200810542162421624718852471
  • KoulmandaMSampathkumarRSBhasinMPrevention of nonimmunologic loss of transplanted islets in monkeysAm J Transplant20141471543155124913821
  • TawaraISunYLewisECAlpha-1-antitrypsin monotherapy reduces graft-versus-host disease after experimental allogeneic bone marrow transplantationProc Natl Acad Sci U S A2012109256456922203983
  • GuttmanOBaranovskiBMSchusterRAcute-phase protein α1-anti-trypsin: diverting injurious innate and adaptive immune responses from non-authentic threatsClin Exp Immunol2015179216117225351931
  • de SerresFBlancoIRole of alpha-1 antitrypsin in human health and diseaseJ Intern Med2014276431133524661570