Related Experiment Video
Updated: Apr 6, 2026

10:16
In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease
Published on: December 20, 2017
8.7K
The multiple facets of alpha-1-antitrypsin.
1Lung Investigation Unit, Queen Elizabeth Hospital Birmingham, Mindelsohn way, Edgbaston, Birmingham B15 2WB, UK.
Annals of Translational Medicine
|July 25, 2015
Summary
Alpha-1-antitrypsin (AAT) is a key proteinase inhibitor. Its deficiency causes lung disease, but AAT also has non-proteolytic functions, suggesting broader therapeutic potential.
Area of Science:
- Biochemistry
- Immunology
- Pulmonology
Background:
- Alpha-1-antitrypsin (AAT) is a primary inhibitor of serine proteinases.
- Genetic deficiency in AAT is linked to neutrophilic diseases, notably severe emphysema, via the proteinase/antiprotease hypothesis.
- Emerging evidence highlights AAT's role in regulating inflammatory and immunological responses beyond its inhibitory functions.
Purpose of the Study:
- To review the established and emerging functions of Alpha-1-antitrypsin (AAT).
- To explore the non-proteolytic, immunomodulatory roles of AAT.
- To discuss the potential of AAT as a therapeutic agent in diverse clinical settings.
Main Methods:
- Comprehensive literature review of published studies on Alpha-1-antitrypsin.
- Analysis of research detailing both traditional proteinase inhibition and novel functions.
- Synthesis of findings related to AAT's role in disease and therapeutic applications.
Main Results:
- AAT effectively inhibits serine proteinases, and its deficiency leads to tissue damage.
- AAT possesses significant non-proteolytic functions influencing inflammatory and immunological processes.
- The multifaceted role of AAT supports its potential as a therapeutic agent.
Conclusions:
- Alpha-1-antitrypsin (AAT) exhibits both traditional proteinase inhibition and novel non-proteolytic functions.
- These diverse roles suggest AAT's therapeutic utility extends to various clinical conditions beyond genetic deficiency diseases.
- Further research into AAT's polyfunctional nature is warranted for clinical applications.
Related Concept Videos
Chronic Obstructive Pulmonary Disease-II: Pathophysiology
5.1K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
Chronic Inflammation
5.1K
Asthma-II: Pathophysiology and Classification
4.9K
Asthma is a prevalent chronic respiratory condition marked by inflammation and hyperresponsiveness of the airways. Its pathophysiology involves complex interactions among inflammatory pathways, immune responses, and neural mechanisms.
Additionally, environmental and genetic factors play crucial roles in determining an individual's susceptibility to asthma and the severity of their condition.
Critical processes in asthma pathophysiology include:
Additionally, environmental and genetic factors play crucial roles in determining an individual's susceptibility to asthma and the severity of their condition.
Critical processes in asthma pathophysiology include:
4.9K
Asthma: Pathogenesis and Management
1.7K
Asthma is a chronic pulmonary condition involving inflammation of the airways, hyper-reactivity, and reversible obstruction of the airways. This condition can significantly impact a person's quality of life, making breathing difficult and leading to distressing symptoms.
Asthma is classified as allergic and non-allergic. Allergens such as dust mites, pollen, and pet dander trigger allergic asthma, while factors like cold air, intense emotions, or exercise can induce non-allergic asthma.
Asthma is classified as allergic and non-allergic. Allergens such as dust mites, pollen, and pet dander trigger allergic asthma, while factors like cold air, intense emotions, or exercise can induce non-allergic asthma.
1.7K
Asthma-IV: Diagnostic and Management
3.3K
The diagnosis and management of asthma are comprehensive, encompassing clinical assessments, lung function tests, and pharmacological interventions. Here's an overview:
Clinical Assessment for Asthma:
This is the first step in diagnosing and managing asthma. It includes:
Clinical Assessment for Asthma:
This is the first step in diagnosing and managing asthma. It includes:
3.3K
Amyloid Fibrils
13.1K
Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
13.1K
Amyloid Fibrils
7.0K
7.0K

