RGD Reference Report - Lung disease associated with alpha1-antitrypsin deficiency. - Rat Genome Database

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Lung disease associated with alpha1-antitrypsin deficiency.

Authors: Tuder, RM  Janciauskiene, SM  Petrache, I 
Citation: Tuder RM, etal., Proc Am Thorac Soc. 2010 Nov;7(6):381-6.
RGD ID: 4892126
Pubmed: PMID:21030517   (View Abstract at PubMed)
PMCID: PMC3136958   (View Article at PubMed Central)
DOI: DOI:10.1513/pats.201002-020AW   (Journal Full-text)

alpha(1)-Antitrypsin (A1AT) is a polyvalent, acute-phase reactant with an extensive range of biological functions that go beyond those usually linked to its antiprotease (serpin) activities. Genetic mutations cause a systemic deficiency of A1AT, leading to liver and pulmonary diseases, including emphysema and chronic bronchitis. The pathogenesis of emphysema, which involves the destruction of small airway structures and alveolar units, is triggered by cigarette smoke and pollutants. The tissue damage caused by these agents is further potentiated by the mutual interactions between apoptosis, oxidative stress, and protease/antiprotease imbalance. These processes lead to the activation of endogenous mediators of tissue destruction, including the lipid ceramide, extracellular matrix proteins, and abnormal inflammatory cell signaling. In this review, we propose that A1AT has a range of actions that are not restricted to protease inhibition but rather extend to mitigate a range of these pathological processes involved in the development of emphysema. We discuss the evidence indicating that A1AT blocks apoptosis by binding and inhibiting active caspase-3 and modulates a broad range of inflammatory responses induced by neutrophils and by lipopolysaccharide and tumor necrosis factor-alpha signaling.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
pulmonary emphysema  IAGP 4892126 RGD 
pulmonary emphysema  ISOSERPINA1 (Homo sapiens)4892126 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Serpina1  (serpin family A member 1)

Genes (Homo sapiens)
SERPINA1  (serpin family A member 1)


Additional Information