Pathology of the Elastic Matrix

Beth A. Kozel, Dirk Hubmacher

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review

1 Scopus citations

Abstract

Developmental elastic ber diseases in humans occur as a result of mutations within several of these genes (Table 2.1), with evidence for both haploinsuciency, that is, reduced amounts of the aected protein, and dominant negative eects, that is, formation of aberrant elastic matrices. In addition, several human conditions have been ascribed to the pathological activation of extracellular proteases that weaken or degrade elastic bers, altering the biomechanical properties of the aected tissues, which ultimately leads to tissue failure. e most commonly aected organ systems are the aorta (resulting in altered arterial caliber and compliance), the lung (causing developmental or acquired emphysema), the skin (leading to alterations in skin mechanics and overly tight or loose skin), urogenital organs (contributing to pelvic organ prolapse or stress urinary incontinence), and joints (resulting in hyperextensible or sti joints). For 2.3.4 LTBP 54 2.3.4.1 LTBP4 (ARCL) 55 2.3.5 ABCC6 (Pseudoxanthoma Elasticum) 56 2.4 Nongenetic, Acquired, and Environmental Diseases Aecting Elastic Matrices 58 2.4.1 Abdominal Aortic Aneurysms 58 2.4.2 Solar Elastosis 59 2.4.3 Aging Processes in Skin and Lung 60 2.4.4 Age-Related Arterial Stiening 61 2.4.5 Pulmonary Emphysema due to ELN Degradation by Neutrophil Elastase and MMPs 62 2.5 Summary and Outlook 63 Acknowledgments 65 References 66 any given gene mutation or nongenetic insult, each tissue may be more or less severely aected, reecting the relative quantity of the aected protein and its relative contribution to the proper function of the respective tissue.

Original languageEnglish
Title of host publicationElastic Fiber Matrices
Subtitle of host publicationBiomimetic Approaches to Regeneration and Repair
PublisherCRC Press
Pages31-80
Number of pages50
ISBN (Electronic)9781498721936
DOIs
StatePublished - 1 Jan 2018
Externally publishedYes

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