| scholarly article | Q13442814 |
| P2093 | author name string | B Gross | |
| J K Stoller | |||
| S Janciauskiene | |||
| L M Bjursten | |||
| M Wencker | |||
| M Grebe | |||
| P2860 | cites work | Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy | Q24554310 |
| Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin | Q28218698 | ||
| The serpin superfamily of proteinase inhibitors: structure, function, and regulation | Q28254893 | ||
| Differential detection of PAS-positive inclusions formed by the Z, Siiyama, and Mmalton variants of alpha1-antitrypsin. | Q30343942 | ||
| Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data | Q30619954 | ||
| Conformational properties of serine proteinase inhibitors (serpins) confer multiple pathophysiological roles | Q34201670 | ||
| Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy | Q34249676 | ||
| Structure and variation of human alpha 1-antitrypsin | Q34277158 | ||
| The Z type variation of human alpha 1-antitrypsin causes a protein folding defect | Q34304294 | ||
| The mechanism of Z alpha 1-antitrypsin accumulation in the liver | Q34440939 | ||
| Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys | Q34993988 | ||
| alpha1-Antitrypsin deficiency . 5: intravenous augmentation therapy: current understanding | Q35536847 | ||
| Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo | Q35755014 | ||
| Emphysema in alpha1-antitrypsin deficiency: does replacement therapy affect outcome? | Q36051352 | ||
| Alpha1-antitrypsin deficiency-associated panniculitis: case report and review of treatment options | Q36178769 | ||
| A review of alpha-1 antitrypsin deficiency | Q36222884 | ||
| Alpha-1-antitrypsin deficiency: optimal therapeutic regimen based on population pharmacokinetics | Q36225322 | ||
| Natural history of alpha-1-protease inhibitor deficiency | Q39646905 | ||
| Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature | Q39683579 | ||
| Molecular pathology of alpha 1-antitrypsin deficiency and its significance to clinical medicine | Q40568842 | ||
| Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema | Q40797789 | ||
| Weber-Christian syndrome associated with an alpha-1 antitrypsin deficiency. Familial investigation | Q40916317 | ||
| The proteinase-antiproteinase theory of emphysema: a speculative analysis of recent advances into the pathogenesis of emphysema | Q41688515 | ||
| Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema | Q42454527 | ||
| Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema | Q42521815 | ||
| Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant | Q47622881 | ||
| Alpha-1-antitrypsin deficiency panniculitis. | Q55346255 | ||
| Systemic nodular panniculitis in a patient with alpha-1 antitrypsin deficiency (PiSS phenotype) | Q59182881 | ||
| Baseline characteristics of enrollees in the National Heart, Lung and Blood Institute Registry of alpha 1-antitrypsin deficiency. Alpha 1-Antitrypsin Deficiency Registry Study Group | Q73092766 | ||
| A case of systemic nodular panniculitis associated with M1 (Val213) Z phenotype of alpha 1-protease inhibitor | Q73382404 | ||
| Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin) | Q78576744 | ||
| P433 | issue | 4 | |
| P304 | page(s) | 370-375 | |
| P577 | publication date | 2009-02-16 | |
| P1433 | published in | Dermatology | Q5262700 |
| P1476 | title | New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy | |
| P478 | volume | 218 |
| Q56243502 | Advances in managing COPD related to α -antitrypsin deficiency: An under-recognized genetic disorder |
| Q64974062 | Alpha-1 Antitrypsin Substitution for Extrapulmonary Conditions in Alpha-1 Antitrypsin Deficient Patients. |
| Q37986292 | Alpha-1 antitrypsin: a potent anti-inflammatory and potential novel therapeutic agent |
| Q38937853 | An ECLIPSE View of Alpha-1 Antitrypsin Deficiency |
| Q38933451 | Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody |
| Q34182669 | Defining the mechanism of polymerization in the serpinopathies |
| Q34980209 | Efficacy of alpha1-antitrypsin augmentation therapy in conditions other than pulmonary emphysema |
| Q35596461 | Identification of a novel SERPINA-1 mutation causing alpha-1 antitrypsin deficiency in a patient with severe bronchiectasis and pulmonary embolism |
| Q42090487 | Liquefying panniculitis associated with intraductal papillary mucinous neoplasm |
| Q33743024 | Long-term clinical outcomes following treatment with alpha 1-proteinase inhibitor for COPD associated with alpha-1 antitrypsin deficiency: a look at the evidence |
| Q38643507 | Neutrophilic panniculitis associated with alpha-1-antitrypsin deficiency: an update |
| Q89907121 | New Patient-Centric Approaches to the Management of Alpha-1 Antitrypsin Deficiency |
| Q60363804 | Pannikulitis des Oberschenkels und Lungenemphysem bei einem 54-jährigen Patienten |
| Q38988633 | Recent advances in understanding and treating COPD related to α1-antitrypsin deficiency |
| Q83710108 | Sustained expression of circulating human alpha-1 antitrypsin reduces inflammation, increases CD4+FoxP3+ Treg cell population and prevents signs of experimental autoimmune encephalomyelitis in mice |
| Q38604630 | The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult |
| Q55011363 | The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals. |
| Q35214289 | Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency |
| Q38092851 | Twenty years of polymers: a personal perspective on 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 |
| Q64047576 | [A rare cause of severe panniculitis] |
| Q37855954 | α(1)-antitrypsin deficiency and inflammation |
| Q34428735 | α1 Antitrypsin deficiency: current best practice in testing and augmentation therapy |
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