| scholarly article | Q13442814 |
| P6179 | Dimensions Publication ID | 1048738506 |
| P356 | DOI | 10.1038/357605A0 |
| P698 | PubMed publication ID | 1608473 |
| P50 | author | David A Lomas | Q62729064 |
| P2093 | author name string | Carrell RW | |
| Evans DL | |||
| Finch JT | |||
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| P433 | issue | 6379 | |
| P407 | language of work or name | English | Q1860 |
| P304 | page(s) | 605-607 | |
| P577 | publication date | 1992-06-01 | |
| P1433 | published in | Nature | Q180445 |
| P1476 | title | The mechanism of Z alpha 1-antitrypsin accumulation in the liver | |
| P478 | volume | 357 |
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| Q42722787 | A missense LAMB2 mutation causes congenital nephrotic syndrome by impairing laminin secretion |
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| Q41135275 | A novel transcription factor regulates expression of the vacuolar H+-ATPase B2 subunit through AP-2 sites during monocytic differentiation |
| Q57707214 | A polymorphism of the alpha1-antitrypsin gene represents a risk factor for liver disease |
| Q31137762 | A redox-sensitive loop regulates plasminogen activator inhibitor type 2 (PAI-2) polymerization |
| Q34005076 | A screen to identify cellular modulators of soluble levels of an amyotrophic lateral sclerosis (ALS)-causing mutant SOD1. |
| Q35994489 | A single-chain variable fragment intrabody prevents intracellular polymerization of Z α1-antitrypsin while allowing its antiproteinase activity |
| Q72042576 | A synthetic peptide which specifically inhibits heat-treated interleukin-8 binding and chemotaxis for neutrophils |
| Q72167590 | A thermostable mutation located at the hydrophobic core of alpha 1-antitrypsin suppresses the folding defect of the Z-type variant |
| Q72402227 | A young woman with cirrhosis: autoimmune hepatitis vs. alpha 1-antitrypsin deficiency |
| Q27932696 | ADD66, a gene involved in the endoplasmic reticulum-associated degradation of alpha-1-antitrypsin-Z in yeast, facilitates proteasome activity and assembly. |
| Q28550962 | AFM Imaging Reveals Topographic Diversity of Wild Type and Z Variant Polymers of Human α1-Proteinase Inhibitor |
| Q38813513 | Aberrant disulphide bonding contributes to the ER retention of alpha1-antitrypsin deficiency variants. |
| Q44235328 | Acid Denaturation of alpha1-antitrypsin: characterization of a novel mechanism of serpin polymerization. |
| Q38281657 | Adult liver disorders caused by inborn errors of metabolism: review and update |
| Q38171382 | Advances in alpha-1-antitrypsin deficiency liver disease |
| Q56243502 | Advances in managing COPD related to α -antitrypsin deficiency: An under-recognized genetic disorder |
| Q34652212 | Aeropin from the extremophile Pyrobaculum aerophilum bypasses the serpin misfolding trap |
| Q42111387 | Aggregated myocilin induces russell bodies and causes apoptosis: implications for the pathogenesis of myocilin-caused primary open-angle glaucoma |
| Q50527105 | Albumin contamination of a purified human alpha 1-antitrypsin preparation does not affect either structural conformation or the electrophoretic mobility of the inhibitor. |
| Q57595961 | All-Atom Simulations Reveal How Single-Point Mutations Promote Serpin Misfolding |
| Q77798394 | Alpha 1-antitrypsin deficiency |
| Q74110117 | Alpha 1-antitrypsin deficiency: memorandum from a WHO meeting |
| Q72823775 | Alpha 1-antitrypsin: a guardian of vascular tissue |
| Q42315252 | Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection |
| Q91762731 | Alpha-1 Antitrypsin Mutations: Is One Too Many? |
| Q33806741 | Alpha-1 antitrypsin Pi*SZ genotype: estimated prevalence and number of SZ subjects worldwide. |
| Q38234419 | Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions |
| Q42924805 | Alpha-1 antitrypsin augmentation therapy decreases miR-199a-5p, miR-598 and miR-320a expression in monocytes via inhibition of NFκB. |
| Q24816929 | Alpha-1 antitrypsin deficiency is not a rare disease but a disease that is rarely diagnosed |
| Q37056312 | Alpha-1 antitrypsin deficiency: a conformational disease associated with lung and liver manifestations. |
| Q38114852 | Alpha-1 antitrypsin deficiency: new developments in augmentation and other therapies |
| Q38092880 | Alpha-1 antitrypsin deficiency: the European experience |
| Q37986292 | Alpha-1 antitrypsin: a potent anti-inflammatory and potential novel therapeutic agent |
| Q35063726 | Alpha-1 proteinase inhibitors for the treatment of alpha-1 antitrypsin deficiency: safety, tolerability, and patient outcomes |
| Q38781367 | Alpha-1-antitrypsin deficiency-related panniculitis: two cases with diverse clinical courses |
| Q34437066 | Alpha-1-antitrypsin deficiency: a new paradigm for hepatocellular carcinoma in genetic liver disease |
| Q41250313 | Alpha-1-antitrypsin deficiency: biochemistry and clinical manifestations |
| Q36846798 | Alpha-1-antitrypsin deficiency: current concepts |
| Q36410546 | Alpha-1-antitrypsin deficiency: diagnosis, pathophysiology, and management |
| Q38060725 | Alpha-1-antitrypsin in pathogenesis of hepatocellular carcinoma |
| Q45138502 | Alpha-1-antitrypsin is produced by human neutrophil granulocytes and their precursors and liberated during granule exocytosis. |
| Q40833512 | Alpha-1-antitrypsin stimulates fibroblast proliferation and procollagen production and activates classical MAP kinase signalling pathways |
| Q28543931 | Alpha-1-antitrypsin: a novel human high temperature requirement protease A1 (HTRA1) substrate in human placental tissue |
| Q33917374 | Alpha-to-beta structural transformation of ovalbumin: heat and pH effects |
| Q93025869 | Alpha1-Antitrypsin Deficiency: Transition of Care for the Child With AAT Deficiency into Adulthood |
| Q30350461 | Alpha1-antitrypsin as model to assess glycan function in endoplasmic reticulum. |
| Q34428996 | Alpha1-antitrypsin deficiency |
| Q35788002 | Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology |
| Q24554310 | Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy |
| Q33842205 | Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization |
| Q35344873 | Altered protein folding may be the molecular basis of most cases of cystic fibrosis |
| Q44799120 | Altered secretion of a TIGR/MYOC mutant lacking the olfactomedin domain |
| Q36619374 | Alveolar macrophage activation and an emphysema-like phenotype in adiponectin-deficient mice |
| Q51651054 | American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. |
| Q38937853 | An ECLIPSE View of Alpha-1 Antitrypsin Deficiency |
| Q47162066 | An RNA structure-mediated, posttranscriptional model of human α-1-antitrypsin expression |
| Q40356915 | An antibody raised against a pathogenic serpin variant induces mutant-like behaviour in the wild-type protein |
| Q37605015 | An antibody that prevents serpin polymerisation acts by inducing a novel allosteric behaviour. |
| Q40900972 | An integrative approach combining ion mobility mass spectrometry, X-ray crystallography, and nuclear magnetic resonance spectroscopy to study the conformational dynamics of α1 -antitrypsin upon ligand binding |
| Q24684030 | An overview of the serpin superfamily |
| Q84382161 | Analysis of surface cavity in serpin family reveals potential binding sites for chemical chaperone to reduce polymerization |
| Q37410570 | Antisense oligonucleotide treatment ameliorates alpha-1 antitrypsin-related liver disease in mice. |
| Q47358621 | Antithrombin-TRI (Ala382 to Thr) causing severe thromboembolic tendency undergoes the S-to-R transition and is associated with a plasma-inactive high-molecular-weight complex of aggregated antithrombin |
| Q24632743 | Arabidopsis AtSerpin1, crystal structure and in vivo interaction with its target protease RESPONSIVE TO DESICCATION-21 (RD21) |
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| Q28213399 | Association between conformational mutations in neuroserpin and onset and severity of dementia |
| Q57909513 | Atomic insights into the genesis of cellular filaments by globular proteins |
| Q37774788 | Augmentation therapy in alpha-1 antitrypsin deficiency: advances and controversies |
| Q37212761 | Autophagic disposal of the aggregation-prone protein that causes liver inflammation and carcinogenesis in alpha-1-antitrypsin deficiency |
| Q37693729 | Autophagy in hepatic fibrosis. |
| Q28485132 | Biochemical characterization of Anopheles gambiae SRPN6, a malaria parasite invasion marker in mosquitoes |
| Q27730918 | Biological implications of a 3 A structure of dimeric antithrombin |
| Q34661105 | Blocking formation of large protein aggregates by small peptides |
| Q38165139 | Broad spectrum of hepatocyte inclusions in humans, animals, and experimental models |
| Q34464961 | C1 inhibitor: molecular and clinical aspects |
| Q41370342 | COOH-terminal substitutions in the serpin C1 inhibitor that cause loop overinsertion and subsequent multimerization |
| Q34288930 | Cataract-causing defect of a mutant γ-crystallin proceeds through an aggregation pathway which bypasses recognition by the α-crystallin chaperone |
| Q31129874 | Causal and synthetic associations of variants in the SERPINA gene cluster with alpha1-antitrypsin serum levels |
| Q58155199 | Cellular processing of the amyloidogenic cystatin C variant of hereditary cerebral hemorrhage with amyloidosis, Icelandic type |
| Q61796986 | Characterisation of a type II functionally-deficient variant of alpha-1-antitrypsin discovered in the general population |
| Q37813223 | Characterisation of serpin polymers in vitro and in vivo. |
| Q38933451 | Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody |
| Q24537406 | Characterization of an ERAD gene as VPS30/ATG6 reveals two alternative and functionally distinct protein quality control pathways: one for soluble Z variant of human alpha-1 proteinase inhibitor (A1PiZ) and another for aggregates of A1PiZ. |
| Q37118167 | Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency |
| Q30540246 | Chopin's serpin |
| Q73330570 | Chronic liver disease in heterozygous alpha1-antitrypsin deficiency PiZ |
| Q24672272 | Cleaved antitrypsin polymers at atomic resolution |
| Q46662744 | Cleaved serpin refolds into the relaxed state via a stressed conformer |
| Q36281062 | Cold rescue of the thermolabile tailspike intermediate at the junction between productive folding and off-pathway aggregation |
| Q33609148 | Collapse of a long axis: single-molecule Förster resonance energy transfer and serpin equilibrium unfolding |
| Q54620011 | Comparison between medium-chain acyl-CoA dehydrogenase mutant proteins overexpressed in bacterial and mammalian cells. |
| Q24795113 | Concentration-dependent effects of native and polymerised alpha1-antitrypsin on primary human monocytes, in vitro |
| Q53333352 | Conformational changes and disease--serpins, prions and Alzheimer's. |
| Q28244193 | Conformational disease |
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| Q36045474 | Conformational properties of the disease-causing Z variant of α1-antitrypsin revealed by theory and experiment |
| Q27652525 | Crystal structure of a stable dimer reveals the molecular basis of serpin polymerization |
| Q27653866 | Crystallographic and Cellular Characterisation of Two Mechanisms Stabilising the Native Fold of α1-Antitrypsin: Implications for Disease and Drug Design |
| Q38264336 | Current status of gene therapy for α-1 antitrypsin deficiency |
| Q72033265 | Defective protein folding as a cause of disease |
| Q36549080 | Deficiency Mutations of Alpha-1 Antitrypsin. Effects on Folding, Function, and Polymerization |
| Q34182669 | Defining the mechanism of polymerization in the serpinopathies |
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| Q36058093 | Determining serpin conformational distributions with single molecule fluorescence |
| Q34467481 | Diagnosis of alpha-1-antitrypsin deficiency in bleeding disorder-related neonatal death |
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| Q27938609 | Differential requirements of novel A1PiZ degradation deficient (ADD)genes in ER-associated protein degradation |
| Q28547169 | Discovery of an inhibitor of Z-alpha1 antitrypsin polymerization |
| Q34394333 | Dissecting glycoprotein quality control in the secretory pathway |
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| Q88932489 | Dynamic changes in complexes of IRE1α, PERK, and ATF6α during endoplasmic reticulum stress |
| Q41891765 | Dysfunctional glycogen storage in a mouse model of alpha1-antitrypsin deficiency |
| Q28235801 | EDEM accelerates ERAD by preventing aberrant dimer formation of misfolded alpha1-antitrypsin |
| Q77696805 | Early steps in the unfolding of thermolysin-like proteases |
| Q43258230 | Effect of citrullination on the function and conformation of antithrombin |
| Q73456677 | Effect of glycosylation on the stability of alpha1-antitrypsin toward urea denaturation and thermal deactivation |
| Q71412784 | Elastase inhibitory capacity of purified canine alpha-1-antitrypsin |
| Q47818629 | Electrophoresis- and FRET-Based Measures of Serpin Polymerization |
| Q30332997 | Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation. |
| Q36432474 | Embelin binds to human neuroserpin and impairs its polymerisation. |
| Q40843065 | Emergence of a stage-dependent human liver disease signature with directed differentiation of alpha-1 antitrypsin-deficient iPS cells |
| Q37780552 | Emerging drugs for alpha-1-antitrypsin deficiency |
| Q37410544 | Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency |
| Q48301788 | Endoplasmic reticulum proteins SDF2 and SDF2L1 act as components of the BiP chaperone cycle to prevent protein aggregation. |
| Q38925531 | Endoplasmic reticulum proteostasis in hepatic steatosis. |
| Q35901528 | Engineering an enzyme to resist boiling |
| Q55221866 | Enhancement of Alpha 1-antitrypsin Production in Pichia pastoris by Designing and Optimizing Medium Using Elemental Analysis. |
| Q30484036 | Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis |
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| Q51636248 | European screening for alpha1 -antitrypsin deficiency in subjects with lung disease. |
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| Q33653261 | Exogenous alpha 1-antitrypsin down-regulates SERPINA1 expression |
| Q90069083 | Exome Sequencing Reveals Immune Genes as Susceptibility Modifiers in Individuals with α1-Antitrypsin Deficiency |
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| Q33291582 | Expression of hereditary hemochromatosis C282Y HFE protein in HEK293 cells activates specific endoplasmic reticulum stress responses |
| Q34138249 | Familial conformational diseases and dementias |
| Q28145741 | Familial dementia caused by polymerization of mutant neuroserpin |
| Q35745998 | Fibrinogen brescia: hepatic endoplasmic reticulum storage and hypofibrinogenemia because of a gamma284 Gly-->Arg mutation |
| Q41664525 | Folding intermediates are involved in genetic diseases? |
| Q53516475 | Functional analysis of novel alpha-1 antitrypsin variants G320R and V321F. |
| Q35094717 | Functional and dysfunctional conformers of human neuroserpin characterized by optical spectroscopies and Molecular Dynamics |
| Q93101948 | Functional characterization of a SNP (F51S) found in human alpha 1-antitrypsin |
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| Q38937862 | Gene Therapy for Alpha-1 Antitrypsin Deficiency Lung Disease |
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| Q24300563 | Grp78, Grp94, and Grp170 interact with alpha1-antitrypsin mutants that are retained in the endoplasmic reticulum |
| Q36073709 | Health implications of alpha1-antitrypsin deficiency in Sub-Sahara African countries and their emigrants in Europe and the New World |
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| Q35040113 | Hepatic manifestations of pulmonary diseases |
| Q34246574 | Hepatic progenitor cell proliferation and liver injury in α-1-antitrypsin deficiency |
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| Q84082534 | Hepatoid carcinoma of the skin: spontaneous rat skin hepatoid carcinoma with eosinophilic globules and crystals immunoreactive to alpha-1-antitrypsin |
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| Q44260056 | In vitro and in silico design of alpha1-antitrypsin mutants with different conformational stabilities |
| Q39085645 | Increased ERK signalling promotes inflammatory signalling in primary airway epithelial cells expressing Z α1-antitrypsin |
| Q35048487 | Increased body temperature accelerates aggregation of the Leu-68-->Gln mutant cystatin C, the amyloid-forming protein in hereditary cystatin C amyloid angiopathy. |
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| Q35156969 | Longer telomere length in COPD patients with α1-antitrypsin deficiency independent of lung function |
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| Q37103683 | National Emphysema Treatment Trial state of the art: genetics of emphysema |
| Q28252760 | Neuroserpin binds Abeta and is a neuroprotective component of amyloid plaques in Alzheimer disease |
| Q38643507 | Neutrophilic panniculitis associated with alpha-1-antitrypsin deficiency: an update |
| Q30392273 | New Concepts in Alpha-1 Antitrypsin Deficiency Disease Mechanisms |
| Q37466723 | New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy |
| Q43135777 | New insight into serpin polymerization and aggregation |
| Q72271908 | Nontransplant options for the treatment of metabolic liver disease: saving livers while saving lives |
| Q33975865 | OS-9 facilitates turnover of nonnative GRP94 marked by hyperglycosylation. |
| Q50522986 | On the molecular structure of human neuroserpin polymers. |
| Q33902839 | Organizational diversity among distinct glycoprotein endoplasmic reticulum-associated degradation programs |
| Q43840170 | Osmolytes as modulators of conformational changes in serpins |
| Q81403027 | Outcome of PiSS and PiSZ alpha-1-antitrypsin deficiency presenting with liver involvement |
| Q51536039 | Oxidation of Z α1-antitrypsin by cigarette smoke induces polymerization: a novel mechanism of early-onset emphysema. |
| Q37343918 | Oxidized {alpha}1-antitrypsin stimulates the release of monocyte chemotactic protein-1 from lung epithelial cells: potential role in emphysema |
| Q64098692 | Parallel transjugular intrahepatic portosystemic shunt with Viatorr stents for primary TIPS insufficiency: Case series and review of literature |
| Q43180243 | Parker B. Francis lectureship. Antitrypsin deficiency, the serpinopathies, and chronic obstructive pulmonary disease |
| Q36545632 | Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency |
| Q57980256 | Pathogenic α1-Antitrypsin Polymers Are Formed by Reactive Loop-β-Sheet A Linkage |
| Q40966808 | Pediatric gastroenterology. Update on metabolic liver disease |
| Q35247414 | PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomes |
| Q28285553 | Plasminogen-activator inhibitor type 2 (PAI-2) is a spontaneously polymerising SERPIN. Biochemical characterisation of the recombinant intracellular and extracellular forms |
| Q39087063 | Polymerization of human angiotensinogen: insights into its structural mechanism and functional significance |
| Q31645732 | Polymerization of plasminogen activator inhibitor-1. |
| Q37203729 | Polymers and inflammation: disease mechanisms of the serpinopathies |
| Q35755014 | Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo |
| Q42521815 | Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema |
| Q35615846 | Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies |
| Q45824312 | Practice and knowledge about diagnosis and treatment of alpha-1 antitrypsin deficiency in Spain and Portugal |
| Q72631383 | Preparation and characterization of latent alpha 1-antitrypsin |
| Q40425487 | Presymptomatic testing for genetic diseases of later life. Pharmacoepidemiological considerations |
| Q38039045 | Prevalence of α1-antitrypsin deficiency alleles PI*S and PI*Z worldwide and effective screening for each of the five phenotypic classes PI*MS, PI*MZ, PI*SS, PI*SZ, and PI*ZZ: a comprehensive review |
| Q27652055 | Preventing serpin aggregation: The molecular mechanism of citrate action upon antitrypsin unfolding |
| Q47640033 | Probing the folding pathway of a consensus serpin using single tryptophan mutants |
| Q42964603 | Probing the local conformational change of alpha1-antitrypsin |
| Q52534363 | Probing the unfolding pathway of alpha1-antitrypsin. |
| Q74012611 | Prognosis of alpha-1-antitrypsin deficiency-related liver disease in the era of paediatric liver transplantation |
| Q37853216 | Proteases as therapeutics |
| Q37845738 | Protein N-glycosylation, protein folding, and protein quality control. |
| Q34815754 | Protein aggregation in disease: a role for folding intermediates forming specific multimeric interactions |
| Q30336514 | Protein fiber linear dichroism for structure determination and kinetics in a low-volume, low-wavelength couette flow cell |
| Q71477938 | Protein folding and calcium binding defects arising from familial hypercholesterolemia mutations of the LDL receptor |
| Q36753974 | Protein misfolding and aggregation in cataract disease and prospects for prevention |
| Q34928474 | Protein misfolding and the serpinopathies |
| Q81231764 | Protein misfolding, aggregation, and degradation in disease |
| Q36432134 | Protein quality control: the who's who, the where's and therapeutic escapes |
| Q90182143 | Protocol for the EARCO Registry: a pan-European observational study in patients with α1-antitrypsin deficiency |
| Q54601648 | Purification and characterization of α1-antitrypsin secreted by recombinant yeast Saccharomyces diastaticus |
| Q39635808 | Purification of alpha-1-antitrypsin monomer by preparative electrophoresis |
| Q35937953 | Quantitation of circulating wild-type alpha-1-antitrypsin in heterozygous carriers of the S and Z deficiency alleles |
| Q37149157 | Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model |
| Q57008825 | Rapid insight into C60 influence on biological functions of proteins |
| Q23911017 | Rationale and design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis. Alpha-1 protocol |
| Q64054667 | Reactive centre loop dynamics and serpin specificity |
| Q43116515 | Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway |
| Q41942630 | Real time PCR detection of the PI*Z and PI*S mutations associated with alpha-1 antitrypsin deficiency |
| Q58786455 | Real world evaluation of a novel lateral flow assay (AlphaKit® QuickScreen) for the detection of alpha-1-antitrypsin deficiency |
| Q63246374 | Recent Developments in mRNA-Based Protein Supplementation Therapy to Target Lung Diseases |
| Q38988633 | Recent advances in understanding and treating COPD related to α1-antitrypsin deficiency |
| Q38111790 | Recent advances in α-1-antitrypsin deficiency-related lung disease |
| Q41006428 | Recent pulmonary observations in alpha 1-antitrypsin deficiency, primary biliary cirrhosis, chronic hepatitis C, and other hepatic problems |
| Q34295276 | Reference and interpretive ranges for α(1)-antitrypsin quantitation by phenotype in adult and pediatric populations |
| Q48097792 | Refolding and Polymerization Pathways of Neuroserpin |
| Q35027539 | Reglucosylation by UDP-glucose:glycoprotein glucosyltransferase 1 delays glycoprotein secretion but not degradation |
| Q53330981 | Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha1-antitrypsin Z in the classical form of alpha1-antitrypsin deficiency. |
| Q42126974 | Retarded protein folding of deficient human alpha 1-antitrypsin D256V and L41P variants |
| Q41509050 | Review: alpha 1-antitrypsin deficiency associated liver disease |
| Q35167911 | Role of Drosophila EDEMs in the degradation of the alpha-1-antitrypsin Z variant |
| Q48068590 | Role of a distal enhancer containing a functional NF-kappa B-binding site in lipopolysaccharide-induced expression of a novel alpha 1-antitrypsin gene |
| Q35594557 | Role of alpha-1-antichymotrypsin deficiency in promoting cirrhosis in two siblings with heterozygous alpha-1-antitrypsin deficiency phenotype SZ. |
| Q37070325 | Roles for proteinases in the pathogenesis of chronic obstructive pulmonary disease |
| Q91971881 | Russell-Like Bodies in Plant Seeds Share Common Features With Prolamin Bodies and Occur Upon Recombinant Protein Production |
| Q30398587 | S-glutathionylation: from molecular mechanisms to health outcomes |
| Q36813713 | SERPINA1 and ELA2 polymorphisms are not associated with COPD or lung cancer |
| Q34636554 | SERPINB11 is a new noninhibitory intracellular serpin. Common single nucleotide polymorphisms in the scaffold impair conformational change |
| Q42673729 | Selenoprotein S/SEPS1 modifies endoplasmic reticulum stress in Z variant alpha1-antitrypsin deficiency |
| Q36438832 | Sequestration of mutated alpha1-antitrypsin into inclusion bodies is a cell-protective mechanism to maintain endoplasmic reticulum function. |
| Q90151818 | Serine protease inhibitors and human wellbeing interplay: new insights for old friends |
| Q35706783 | Serpin Inhibition Mechanism: A Delicate Balance between Native Metastable State and Polymerization. |
| Q36279544 | Serpin alpha 1proteinase inhibitor probed by intrinsic tryptophan fluorescence spectroscopy |
| Q52597535 | Serpin functions in host-pathogen interactions. |
| Q27640477 | Serpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerol |
| Q34932013 | Serpinopathies and the conformational dementias |
| Q24597208 | Serpins flex their muscle: II. Structural insights into target peptidase recognition, polymerization, and transport functions |
| Q37222018 | Serpins in T cell immunity |
| Q42790048 | Serpins show structural basis for oligomer toxicity and amyloid ubiquity. |
| Q57079410 | Serpins' mystery solved |
| Q40704381 | Serpins: the uncut version |
| Q34325994 | Severe alpha-1 antitrypsin deficiency in composite heterozygotes inheriting a new splicing mutation QOMadrid |
| Q35746195 | Simultaneous phenotyping and quantification of α-1-antitrypsin by liquid chromatography-tandem mass spectrometry |
| Q37249690 | Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency |
| Q24645055 | Small molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregates |
| Q39899143 | Small-molecule peptides inhibit Z alpha1-antitrypsin polymerization |
| Q37282499 | Smoothing a rugged protein folding landscape by sequence-based redesign |
| Q35096681 | Spn1 regulates the GNBP3-dependent Toll signaling pathway in Drosophila melanogaster |
| Q34058519 | Spontaneous assembly of a self-complementary oligopeptide to form a stable macroscopic membrane |
| Q34876052 | Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytes |
| Q45735547 | Stable therapeutic serum levels of human alpha-1 antitrypsin (AAT) after portal vein injection of recombinant adeno-associated virus (rAAV) vectors |
| Q35923379 | Stem cell-based therapy for α₁-antitrypsin deficiency |
| Q35367680 | Structural and functional characterization of cleavage and inactivation of human serine protease inhibitors by the bacterial SPATE protease EspPα from enterohemorrhagic E. coli |
| Q42152423 | Structural dynamics associated with intermediate formation in an archetypal conformational disease. |
| Q71745939 | Structural explanation for the deficiency of S alpha 1-antitrypsin |
| Q45873139 | Survival Advantage of Both Human Hepatocyte Xenografts and Genome-Edited Hepatocytes for Treatment of α-1 Antitrypsin Deficiency. |
| Q42029205 | Sustained miRNA-mediated knockdown of mutant AAT with simultaneous augmentation of wild-type AAT has minimal effect on global liver miRNA profiles. |
| Q37340178 | Targeted treatment in COPD: a multi-system approach for a multi-system disease |
| Q44478799 | Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease |
| Q53547782 | Tauroursodeoxycholic acid inhibits apoptosis induced by Z alpha-1 antitrypsin via inhibition of Bad. |
| Q56338497 | The 1.5 Å Crystal Structure of a Prokaryote Serpin |
| Q91853562 | The Human Cytomegalovirus Nonstructural Glycoprotein UL148 Reorganizes the Endoplasmic Reticulum |
| Q51465407 | The Role of Neutrophils in Alpha-1 Antitrypsin Deficiency. |
| Q35236899 | The Z mutation alters the global structural dynamics of α1-antitrypsin |
| Q34304294 | The Z type variation of human alpha 1-antitrypsin causes a protein folding defect |
| Q27618622 | The active conformation of plasminogen activator inhibitor 1, a target for drugs to control fibrinolysis and cell adhesion |
| Q35579793 | The aggregation-prone intracellular serpin SRP-2 fails to transit the ER in Caenorhabditis elegans |
| Q28186254 | The cerebral proteopathies: neurodegenerative disorders of protein conformation and assembly |
| Q36594740 | The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment |
| Q27015793 | The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology |
| Q37403714 | The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin |
| Q36466323 | The endosomal protein-sorting receptor sortilin has a role in trafficking α-1 antitrypsin |
| Q24791708 | The genetics of chronic obstructive pulmonary disease |
| Q34375148 | The high resolution crystal structure of a native thermostable serpin reveals the complex mechanism underpinning the stressed to relaxed transition |
| Q36519309 | The human serpin proteinase inhibitor-9 self-associates at physiological temperatures |
| Q55011363 | The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals. |
| Q37976265 | The interplay between endoplasmic reticulum stress and inflammation. |
| Q36663487 | The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB. |
| Q71958793 | The liver in adolescents with α-antitrypsin deficiency |
| Q58023315 | The loss of tryptophan 194 in antichymotrypsin lowers the kinetic barrier to misfolding |
| Q42596259 | The mechanism of fibril formation of a non-inhibitory serpin ovalbumin revealed by the identification of amyloidogenic core regions |
| Q41075465 | The molecular basis of alpha 1-antichymotrypsin deficiency in a heterozygote with liver and lung disease |
| Q28191029 | The molecular interactions of heat shock protein 47 (Hsp47) and their implications for collagen biosynthesis |
| Q34610856 | The necrotic gene in Drosophila corresponds to one of a cluster of three serpin transcripts mapping at 43A1.2. |
| Q37362027 | The parallel lives of alpha1-antitrypsin deficiency and pulmonary alveolar proteinosis |
| Q41066319 | The pathological Trento variant of alpha-1-antitrypsin (E75V) shows nonclassical behaviour during polymerization |
| Q36966036 | The promise of gene therapy for the treatment of alpha-1 antitrypsin deficiency |
| Q34256009 | The role of bronchial epithelial cells in the pathogenesis of COPD in Z-alpha-1 antitrypsin deficiency |
| Q37894093 | The role of proteases, endoplasmic reticulum stress and SERPINA1 heterozygosity in lung disease and α-1 anti-trypsin deficiency |
| Q34575654 | The roles of helix I and strand 5A in the folding, function and misfolding of α1-antitrypsin |
| Q36378692 | The selective advantage of alpha1-antitrypsin deficiency |
| Q44590833 | The selective inhibition of serpin aggregation by the molecular chaperone, alpha-crystallin, indicates a nucleation-dependent specificity |
| Q34424129 | The shapes of Z-α1-antitrypsin polymers in solution support the C-terminal domain-swap mechanism of polymerization. |
| Q73047927 | The spontaneous polymerization of plasminogen activator inhibitor type-2 and Z-antitrypsin are due to different molecular aberrations |
| Q24635283 | The structural diversity in α1-antitrypsin misfolding |
| Q34193142 | The tempered polymerization of human neuroserpin |
| Q85118779 | The ubiquitin ligase Hrd1 promotes degradation of the Z variant alpha 1-antitrypsin and increases its solubility |
| Q51736555 | Therapeutic Genome Editing With CRISPR/Cas9 in a Humanized Mouse Model Ameliorates α1-antitrypsin Deficiency Phenotype. |
| Q27675947 | Therapeutic target-site variability in α1-antitrypsin characterized at high resolution |
| Q35214289 | Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency |
| Q35898478 | Three missense variants of metabolic syndrome-related genes are associated with alpha-1 antitrypsin levels |
| Q34313851 | Three new alpha1-antitrypsin deficiency variants help to define a C-terminal region regulating conformational change and polymerization. |
| Q34067153 | Toward development of a screen to identify randomly encoded, foldable sequences. |
| Q64081306 | Transcriptomic Analysis of Mutant Mice Reveals Differentially Expressed Genes and Altered Mechanisms in Both Blood and Brain |
| Q33649874 | Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review |
| Q38092851 | Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency |
| Q44605777 | Two alanines juxtaposed to aggrecan's G1 domain alter its intracellular localization |
| Q42183296 | Two latent and two hyperstable polymeric forms of human neuroserpin |
| Q35807714 | Two-dimensional IR spectroscopy and segmental 13C labeling reveals the domain structure of human γD-crystallin amyloid fibrils |
| Q33553536 | Ubiquitin ligase SYVN1/HRD1 facilitates degradation of the SERPINA1 Z variant/α-1-antitrypsin Z variant via SQSTM1/p62-dependent selective autophagy |
| Q37516657 | Understanding Lung Deposition of Alpha-1 Antitrypsin in Acute Experimental Mouse Lung Injury Model Using Fluorescence Microscopy |
| Q37882046 | Unravelling the twists and turns of the serpinopathies. |
| Q21144995 | Uptake of the necrotic serpin in Drosophila melanogaster via the lipophorin receptor-1 |
| Q41894323 | Using antisense technology to develop a novel therapy for α-1 antitrypsin deficient (AATD) liver disease and to model AATD lung disease |
| Q33550630 | V2R mutations and nephrogenic diabetes insipidus. |
| Q34058884 | What do dysfunctional serpins tell us about molecular mobility and disease? |
| Q36602296 | What we owe to alpha(1)-antitrypsin and to Carl-Bertil Laurell |
| Q71958857 | Wherefore art thou liver disease associated with alpha- 1 antitrypsin deficiency? |
| Q35233352 | Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis |
| Q24550849 | X-ray crystal structure of MENT: evidence for functional loop-sheet polymers in chromatin condensation |
| Q59054588 | Z and the insoluble answer |
| Q33901639 | Z α1-antitrypsin confers a proinflammatory phenotype that contributes to chronic obstructive pulmonary disease |
| Q49046548 | alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization. |
| Q24678727 | alpha1-Antitrypsin deficiency . 6: new and emerging treatments for alpha1-antitrypsin deficiency |
| Q37474870 | alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies |
| Q37855954 | α(1)-antitrypsin deficiency and inflammation |
| Q24633462 | α-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8 |
| Q35115856 | α-1-antitrypsin variants and the proteinase/antiproteinase imbalance in chronic obstructive pulmonary disease |
| Q51481224 | α1-Antitrypsin deficiency. |
| Q37643457 | α1-antitrypsin Deficiency: A Misfolded Secretory Protein Variant with Unique Effects on the Endoplasmic Reticulum |
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