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Quantifying Tissue-Specific Proteostatic Decline in Caenorhabditis elegans

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Protein misfolding and obstructive lung disease.

Catherine M Greene¹, Noel G McElvaney

¹Respiratory Research Division, Dept. Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland. cmgreene@rcsi.ie

Proceedings of the American Thoracic Society

|October 30, 2010

Summary

Misfolded proteins in alpha-1 antitrypsin (A1AT) and alpha-1 antichymotrypsin (ACT) deficiency cause ER stress and inflammation, contributing to lung disease. Therapeutic strategies targeting misfolded protein production are being explored.

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Assays for the Degradation of Misfolded Proteins in Cells

Assays for the Degradation of Misfolded Proteins in Cells

Published on: August 28, 2016

Area of Science:

Cellular Biology
Molecular Medicine
Pulmonology

Background:

Endoplasmic reticulum (ER) stress arises from misfolded proteins, leading to cellular dysfunction.
Alpha-1 antitrypsin (A1AT) and alpha-1 antichymotrypsin (ACT) deficiencies, linked to chronic obstructive lung disease, involve protein misfolding.
Traditionally, lung disease was attributed to antiprotease loss, while liver disease stemmed from protein gain-of-function toxicity.

Purpose of the Study:

To elucidate cellular mechanisms of ER stress in A1AT and ACT deficiencies.
To investigate the link between ER stress and inflammation in lung diseases.
To outline potential therapeutic strategies targeting misfolded protein production.

Main Methods:

Review of cellular mechanisms involved in managing misfolded proteins within the ER.
Analysis of how ER stress activates inflammatory pathways in lung cells.
Exploration of therapeutic interventions for A1AT and ACT deficiencies.

Main Results:

Misfolded proteins in A1AT and ACT deficiencies can cause a toxic gain of function, promoting ER stress.
ER stress is increasingly recognized in lung cells, contributing to an inflammatory phenotype.
These inflammatory responses exacerbate the proteolytic burden in lung diseases.

Conclusions:

The paradigm of A1AT and ACT deficiency-related lung disease is shifting towards including ER stress and inflammation.
Cellular mechanisms coping with misfolded proteins are crucial in these conditions.
Targeting misfolded protein production offers a promising therapeutic avenue.