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Amyloid Fibrils

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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.
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There are various classifications for PH, each relating to different underlying causes and also...

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Refined Murine Model of Idiopathic Pulmonary Fibrosis

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Pulmonary Amyloidosis.

Stefano Levra^1,2, Marcel Opitz³, Francesco Bonella⁴

¹Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.

Seminars in Respiratory and Critical Care Medicine

|October 14, 2025

Summary

This summary is machine-generated.

Amyloidosis involves protein buildup in tissues, with lung involvement being common but often mild. This review covers recent molecular insights, classifications, lung amyloidosis types, and treatments, stressing the need for awareness and specialized centers.

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Area of Science:

Pulmonary Medicine
Rare Diseases
Molecular Biology

Background:

Amyloidosis comprises rare diseases from misfolded protein deposition.
Lung involvement is frequent but typically mild, though severe forms exist.
Understanding molecular mechanisms and classification is evolving.

Purpose of the Study:

To review recent advances in amyloidosis molecular mechanisms and classification.
To describe various forms of lung amyloidosis.
To outline current treatment strategies for lung amyloidosis.

Main Methods:

Literature review of recent scientific advances.
Synthesis of information on molecular mechanisms.
Compilation of data on classification and clinical presentation.
Summary of therapeutic options.

Main Results:

Recent progress in understanding amyloidosis molecular pathways.
Updated classification systems for amyloidosis.
Description of diverse lung amyloidosis manifestations.
Overview of available and emerging treatments.

Conclusions:

Increased medical awareness of amyloidosis is crucial.
Establishing networks of referral centers is vital for patient management.
Timely diagnosis and access to specialized care improve outcomes for lung amyloidosis.