Original contributionMorphogenesis of abnormal elastic fibers in lungs of patients with panacinar and centriacinar emphysema
References (30)
- et al.
Ultrastructural alterations in collagen associated with microscopic foci of human emphysema
Hum Pathol
(1977) - et al.
Histogenesis of unique elastinophilic fibers of elastofibroma: Ultrastructural and immunohistochemical studies
Hum Pathol
(1987) Connective tissue and lymphatics
Pathogenesis of “fibrosis” in interstitial pneumonia: An electron microscopic study
Hum Pathol
(1985)- et al.
A significant part of macrophage-derived growth factor consists of at least two forms of PDGF
Cell
(1985) Committee of Diagnostic Standards for Nontuberculous Respiratory Diseases: Chronic bronchitis, asthma, and pulmonary emphysema
Am Rev Respir Dis
(1962)- et al.
Lung and mediastinum
Chronic Airflow Obstruction in Lung Disease
(1976)Studies in alpha-1-antitrypsin deficiency
Acta Med Scand
(1965)- et al.
Evaluation of the protease-antiprotease theory of human destructive lung disease
Bull Eu Physiopathol Resp
(1980)
The induction of emphysema with elastase. II. Changes in connective tissue
Lab Invest
Ultrastructural studies of elastase-induced experimental emphysema
Jpn J Exp Med
Ultrastructural changes in hamster lung four hours to twenty-four days after exposure to elastase
Anat Rec
Elastic tissue of normal and emphysematous lungs: A tridimensional histological study
Am J Pathol
The development of alveolar septa in fetal sheep lung: An ultrastructural and immunohistochemical study
Am J Anat
Cited by (72)
Decreased Expression of EC-SOD and Fibulin-5 in Alveolar Walls of Lungs From COPD Patients
2022, Archivos de BronconeumologiaCitation Excerpt :Damage consisted in frayed elastic fibres clumped into dense masses lying within remnants of collapsing alveolar walls, coinciding with pathologic changes described by Robert Wright almost sixty years ago28 When interstitium of collapsing walls were further analyzed through TEM, we observed a loss of fibrillary component, mainly in the core of the elastic fibres, together with the presence of vacuoles affecting the amorphous component. A similar pierced pattern has been described in previous TEM studies in humans and murine models of emphysema,29–31 and underpins the importance of structural abnormalities of elastic fibres in areas of emphysematous destruction. Our results showed that lungs from COPD patients exhibit lower expression of EC-SOD in alveolar walls likely leading to an impaired antioxidant defence in these patients.
Extracellular matrix in lung development, homeostasis and disease
2018, Matrix BiologyCitation Excerpt :Cross-sectional studies albeit with small sample sizes consistently show an increase in collagen content and altered fibril morphology in lungs of patients with moderate and severe COPD [112–114]. Abnormal elastin fibers (fragmented, clumped) with variable changes in elastin content are evident in emphysematous lungs [112,113,115,116]. Animal models of emphysema, typically involving airway elastase instillation or chronic cigarette smoke exposure, also demonstrate increased collagen and elastin synthesis with matrix deposition during the establishment and progression of the airspace lesion [117–121].
Does lysozyme play a role in the pathogenesis of COPD?
2015, Medical HypothesesCitation Excerpt :One of the poorly understood features of COPD concerns the observed reduction in elastolysis as the disease becomes more severe [19]. While it has been proposed that this finding may be due to the eventual exhaustion of the elastolytic process, several studies suggest that human emphysematous lungs still have large amounts of elastic fibers [20–23]. Consequently, there should not be a significant reduction in elastolysis.
A cytokine axis regulates elastin formation and degradation
2013, Matrix BiologyCitation Excerpt :Steady state, basal levels of elastin expression and breakdown remain low in adult tissues as well as in cells isolated from adult tissues (Starcher, 1986; Parks et al., 1988). Elastin expression is reactivated in diseases such as pulmonary hypertension, dermal elastosis, pseudoxanthoma elasticum, Buschke–Ollendorff syndrome, Moyamoya disease, cigarette smoke-induced emphysema, severe chronic obstructive pulmonary disease (COPD) and forms of progeria (Holbrook and Byers, 1982; Sephel et al., 1988; Botney et al., 1992; Bernstein et al., 1994; Stenmark et al., 1994; Yamamoto et al., 1997; Hoff et al., 1999; Deslee et al., 2009; Rangasamy et al., 2009), although in many of these disorders the elastin produced is disorganized and dysfunctional (Kuhn et al., 1976; Fukuda et al., 1989; Hoff et al., 1999). Much is known about the molecular mechanisms underlying elastin formation in development and disease.
Pulmonary fibroblasts from COPD patients show an impaired response of elastin synthesis to TGF-β1
2011, Respiratory Physiology and NeurobiologyCitation Excerpt :Several experimental studies have reported that elastin synthesis is increased in emphysematous lungs in animal models (Chambers and Laurent, 1996). Similarly, investigations on human emphysematous lungs report increased synthesis of elastin (Fukuda et al., 1989). In a recent study, Deslee et al. (2009) reported that elastin mRNA expression was significantly increased in the alveolar walls in very severe COPD compared to donors, non-COPD and stages 2–3 COPD), but elastic fibre content was not increased per lung volume.