Maturation of the lung extracellular matrix (ECM) plays an important role in the formation of alveolar gas exchange units. A key step in ECM maturation is cross-linking of collagen and elastin, which imparts stability and functionality to the ECM. During aberrant late lung development in bronchopulmonary dysplasia (BPD) patients and animal models of BPD, alveolarization is blocked, and the func...

BACKGROUND Development of emphysema and vascular stiffness in chronic obstructive pulmonary disease (COPD) may be due to a common mechanism of susceptibility to pulmonary and systemic elastin degradation. OBJECTIVES To investigate whether patients with COPD have evidence of systemic elastin degradation in the skin. METHODS The authors measured cutaneous elastin degradation using immunohisto...

BACKGROUND Elastin fibre detection could be a simple and reliable marker of ventilator associated pneumonia. To confirm this, a prospective study was undertaken to evaluate the diagnostic yield of elastin fibre detection in the diagnosis of ventilator associated pneumonia. METHODS Seventy eight mechanically ventilated patients were evaluated by examining endotracheal aspirates for the presenc...

The fine distribution of the extracellular matrix glycoprotein emilin (previously known as glycoprotein gp115) (Bressan, G. M., I. Castellani, A. Colombatti, and D. Volpin. 1983. J. Biol. Chem. 258: 13262-13267) has been studied at the ultrastructural level with specific antibodies. In newborn chick aorta the protein was exclusively found within elastic fibers. In both post- and pre-embedding i...

Young pigs raised on a copper-deficient diet develop severe abnormalities of connective tissue due to defective cross-linking of collagen and elastin. They eventually succumb to anemia and cardiovascular damage, the latter apparently due to the defective connective tissue metabolism. We evaluated the effects of nutritional copper deficiency upon collagen and elastin synthesis using short-term e...

Glucocorticoid treatment of fibroblasts from late gestation fetal bovine ligamentum nuchae resulted in a time- and dose-dependent selective increase in elastin production. Tropoelastin levels increased 2-3-fold in the presence of 10 nM dexamethasone while total protein synthesis and the rate of cell division decreased with glucocorticoid exposure. Two tropoelastin bands of molecular weights 64,...

The content of elastic tissue has been evaluated in 171 primary breast carcinomas. Of the tumors, 35% had no or very little elastic tissue in the malignant areas, 42% presented with medium elastosis, and 22% had gross elastosis. The occurrence of elastin has been related to different prognostic factors. An increasing amount of elastin was found with increasing amounts of estrogen receptor (p = ...

Human elastases have been identified within serine, cysteine and metallopeptidase families. These enzymes are able to adsorb rapidly onto elastin, but they can also bind onto cell surface-associated proteins such as heparan sulfate proteoglycans, both interactions involving enzyme exosites distinct form active site. Immobilization of elastin at the cell surface will create a sequestered microen...

A modified version of the enzyme-linked immunosorbent assay (ELISA), utilising human insoluble aortic elastin, was used for determination of anti-elastin antibodies in serum from normal and atherosclerotic subjects. The age-related changes in their level among healthy persons were investigated. Anti-elastin antibodies were found in all the tested human sera, showing the highest level at the age...

Williams-Beuren syndrome (WBS) is a microdeletion disorder caused by heterozygous loss of approximately 1.5-Mb pairs of DNA from chromosome 7. Patients with WBS have a characteristic constellation of medical and cognitive findings, with a hallmark feature of generalized arteriopathy presenting as stenoses of elastic arteries and hypertension. Human and mouse studies establish that defects in th...