Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema

scientific article published on May 1998

Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1165/AJRCMB.18.5.3065
P698PubMed publication ID9569237
P5875ResearchGate publication ID13711729

P2093author name stringLomas DA
Elliott PR
Bilton D
P2860cites workInhibitory conformation of the reactive loop of alpha 1-antitrypsinQ27733292
The native strains in the hydrophobic core and flexible reactive loop of a serine protease inhibitor: crystal structure of an uncleaved alpha1-antitrypsin at 2.7 AQ27733963
What do dysfunctional serpins tell us about molecular mobility and disease?Q34058884
Human alpha 1-proteinase inhibitor. Crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for functionQ34255957
The Z type variation of human alpha 1-antitrypsin causes a protein folding defectQ34304294
The mechanism of Z alpha 1-antitrypsin accumulation in the liverQ34440939
Implications of the three-dimensional structure of alpha 1-antitrypsin for structure and function of serpinsQ34515383
Serpin alpha 1proteinase inhibitor probed by intrinsic tryptophan fluorescence spectroscopyQ36279544
New insights into the structural basis of alpha 1-antitrypsin deficiencyQ41285548
Structural transition of alpha 1-antitrypsin by a peptide sequentially similar to beta-strand s4A.Q44020077
Mutations which impede loop/sheet polymerization enhance the secretion of human alpha 1-antitrypsin deficiency variants.Q49057165
P433issue5
P304page(s)670-674
P577publication date1998-05-01
P1433published inAmerican Journal of Respiratory Cell and Molecular BiologyQ4744268
P1476titleLung polymers in Z alpha1-antitrypsin deficiency-related emphysema
P478volume18

Reverse relations

cites work (P2860)
Q423243811H, 15N and 13C backbone resonance assignments of the archetypal serpin α1-antitrypsin
Q36894255Acute exacerbations of chronic bronchitis in elderly patients: pathogenesis, diagnosis and management
Q37056312Alpha-1 antitrypsin deficiency: a conformational disease associated with lung and liver manifestations.
Q36846798Alpha-1-antitrypsin deficiency: current concepts
Q34428996Alpha1-antitrypsin deficiency
Q24678705Alpha1-antitrypsin deficiency. 2: genetic aspects of alpha(1)-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk
Q35788002Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology
Q24554310Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy
Q33842205Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization
Q38937853An ECLIPSE View of Alpha-1 Antitrypsin Deficiency
Q37774788Augmentation therapy in alpha-1 antitrypsin deficiency: advances and controversies
Q38109518Autophagy: a critical regulator of cellular metabolism and homeostasis
Q91386795Calcium signalling in mammalian cell lines expressing wild type and mutant human α1-Antitrypsin
Q36084447Cell and gene therapy for genetic diseases: inherited disorders affecting the lung and those mimicking sudden infant death syndrome
Q38933451Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody
Q37118167Chemical 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
Q24672272Cleaved antitrypsin polymers at atomic resolution
Q46662744Cleaved serpin refolds into the relaxed state via a stressed conformer
Q24795113Concentration-dependent effects of native and polymerised alpha1-antitrypsin on primary human monocytes, in vitro
Q36045474Conformational properties of the disease-causing Z variant of α1-antitrypsin revealed by theory and experiment
Q74148034Detection of circulating and endothelial cell polymers of Z and wild type alpha 1-antitrypsin by a monoclonal antibody
Q28547169Discovery of an inhibitor of Z-alpha1 antitrypsin polymerization
Q35973238Effect of cigarette smoke exposure and structural modifications on the α-1 Antitrypsin interaction with caspases
Q36661493Endotoxin receptor CD14 in PiZ alpha-1-antitrypsin deficiency individuals
Q36444154Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy.
Q57980231Identification of a 4-mer Peptide Inhibitor that Effectively Blocks the Polymerization of Pathogenic Z α1-Antitrypsin
Q39085645Increased ERK signalling promotes inflammatory signalling in primary airway epithelial cells expressing Z α1-antitrypsin
Q33880491Inflammatory cytokine response to exercise in alpha-1-antitrypsin deficient COPD patients 'on' or 'off' augmentation therapy.
Q46718946Inhaled corticosteroids as additional treatment in alpha-1-antitrypsin-deficiency-related COPD.
Q38912052Inhibitory serpins. New insights into their folding, polymerization, regulation and clearance
Q37341652Intracellular and extracellular serpins modulate lung disease
Q36183894Neutrophilic inflammation and IL-8 levels in induced sputum of alpha-1-antitrypsin PiMZ subjects
Q37466723New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy
Q51536039Oxidation of Z α1-antitrypsin by cigarette smoke induces polymerization: a novel mechanism of early-onset emphysema.
Q37343918Oxidized {alpha}1-antitrypsin stimulates the release of monocyte chemotactic protein-1 from lung epithelial cells: potential role in emphysema
Q43180243Parker B. Francis lectureship. Antitrypsin deficiency, the serpinopathies, and chronic obstructive pulmonary disease
Q37203729Polymers and inflammation: disease mechanisms of the serpinopathies
Q35755014Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo
Q42521815Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema
Q35615846Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies
Q34450903Presence of C1-inhibitor polymers in a subset of patients suffering from hereditary angioedema
Q34928474Protein misfolding and the serpinopathies
Q42790541Proteostasis: a new therapeutic paradigm for pulmonary disease
Q38111790Recent advances in α-1-antitrypsin deficiency-related lung disease
Q21284414SP-A binds alpha1-antitrypsin in vitro and reduces the association rate constant for neutrophil elastase.
Q33760369Serine proteinase inhibitor therapy in alpha(1)-antitrypsin inhibitor deficiency and cystic fibrosis
Q36388052The emerging importance of autophagy in pulmonary diseases
Q55011363The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals.
Q41066319The pathological Trento variant of alpha-1-antitrypsin (E75V) shows nonclassical behaviour during polymerization
Q34256009The role of bronchial epithelial cells in the pathogenesis of COPD in Z-alpha-1 antitrypsin deficiency
Q36378692The selective advantage of alpha1-antitrypsin deficiency
Q35214289Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency
Q38092851Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency
Q35233352Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis
Q24678727alpha1-Antitrypsin deficiency . 6: new and emerging treatments for alpha1-antitrypsin deficiency
Q37474870alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies
Q35115856α-1-antitrypsin variants and the proteinase/antiproteinase imbalance in chronic obstructive pulmonary disease
Q51481224α1-Antitrypsin deficiency.

Search more.