Disease relevance of Elastic Tissue

• Decreased functional alpha 1-protease inhibitor, an inhibitor of neutrophil elastase, might render smokers susceptible to elastase-catalyzed destruction of pulmonary elastic fibers and the development of emphysema [1].
• In any case immunohistochemical staining for SAP is useful in the diagnosis of amyloid, in investigation of glomerulonephritis, and in studying disorders of elastic tissue [2].
• In this report, we present evidence that accumulation of dermatan sulfate but not heparan sulfate moieties is linked to impaired elastic fiber assembly that, in turn, contributes substantially to the development of the clinical phenotype in Hurler disease [3].
• Assuming equal translational activity of the control and cutis laxa mRNAs, the reduced mRNA levels could result in diminished elastin production, providing an explanation for the paucity of elastic fibers in the skin and other tissues in cutis laxa [4].
• Severe airflow obstruction, most commonly due to pulmonary emphysema secondary to the destruction of pulmonary elastic tissue, may develop at an early age in persons with alpha 1-AT deficiency [5].

High impact information on Elastic Tissue

• Thus elastin polymer deposition is a crucial aspect of elastic fiber maintenance and is dependent on LOXL1, which serves both as a cross-linking enzyme and an element of the scaffold to ensure spatially defined deposition of elastin [6].
• Elastic fibers are components of the extracellular matrix and confer resilience [6].
• The fibrillin gene encodes a protein in the extracellular matrix, and this protein is widely distributed in elastic tissues [7].
• The distribution of fibronectin was distinct from that of collagen and elastic fibers, but was very similar to reticulin, as demonstrated by conventional histological staining [8].
• Marked longevity of human lung parenchymal elastic fibers deduced from prevalence of D-aspartate and nuclear weapons-related radiocarbon [9].

Chemical compound and disease context of Elastic Tissue

• These results indicate that a serine proteinase functions as a major elastase in granulomatous tissue remodeling and may account for the disappearance of elastic fibers and other elements of the matrix in fully developed granulomas [10].
• Oral submucous fibrosis biopsies exhibited heavily packed aldehyde fuchsin-positive fibers (i.e. elastic fibers) in the submucosa under the light microscope [11].
• Specific observations: 1) accumulation of hyaluronic acid around vessels occurred uniquely in polyps and 2) transverse orientation of elastic fibers was more often seen in vocal fold nodules [12].
• MMP-9 not only participates in the degradation of elastic tissue but also is associated with intimal hyperplasia, subsequent luminal narrowing, and neoangiogenesis [13].
• Current uses of orcein to demonstrate elastic fibers and, following permanganate oxidation (Shikata's modification), hepatitis B surface antigen, copper associated protein, and sulfated mucins, are reviewed [14].

Biological context of Elastic Tissue

• Targeted mutation of the mouse fibrillin-1 gene has recently suggested that deficiency of fibrillin-1 reduces tissue homeostasis rather than elastic fiber formation [15].
• These results are not consistent with the phenotypes of TGF-beta knockout mice or mice with knockouts of other elastic fiber proteins, implying that LTBP-2 performs a yet undiscovered function in early development, perhaps in implantation [16].
• The major physiological role of the serine protease inhibitor alpha 1-antitrypsin (alpha 1-AT) is to protect elastic fibers in the lung from excessive hydrolysis by neutrophil elastase [17].
• These data indicate that RARgamma is required for the formation of normal alveoli and alveolar elastic fibers in the mouse, and that RAR/RXR heterodimers are involved in alveolar morphogenesis [18].
• The increased stiffening is believed to originate from a gradual mechanical senescence of the elastic network, alterations in cross-linking of extracellular matrix components, fibrosis and calcification of elastic fibers (medial elastocalcinosis) [19].

Anatomical context of Elastic Tissue

• Fetal bovine ligamentum nuchae fibroblasts maintained in culture synthesized soluble elastin but were unable to form the insoluble elastic fiber [20].
• The ruthenium red-positive granules were intimately associated with the plasma membrane of intimal smooth muscle cells and attached to collagen fibrils and elastic fibers [21].
• The extracellular microfibril, 10-14 nm in diameter, performs a number of functions, including serving as the scaffolding for deposition of tropoelastin to form elastic fibers [22].
• The extracellular matrix protein fibulin-1 is a distinct component of vessel walls and can be associated with other ligands present in basement membranes, microfibrils, and elastic fibers [23].
• Microfibril-associated glycoprotein (MAGP) is an integral component of microfibrillar structures that play a critical role in the organization of elastic fibers in the extracellular matrix [24].

Associations of Elastic Tissue with chemical compounds

• Striking ultrastructural alterations of collagenous and elastic fibers of the aortic media, which were additive to the effects of BAPN alone, were induced by BAPN and hydralazine [25].
• A variant of the chondroitin sulfate proteoglycan versican, V3, which lacks chondroitin sulfate, has recently been shown to stimulate elastic fiber assembly and decrease proliferation when expressed by retroviral transduction in arterial smooth muscle cells [26].
• Our data suggest that dermatan sulfate-bearing moieties bind to and cause functional inactivation of the 67-kd elastin-binding protein, a molecular chaperone for tropoelastin, which normally facilitates its secretion and assembly into elastic fibers [3].
• By regulating these key interactions, heparin can profoundly influence microfibril and elastic fiber assembly [27].
• A permanganate-high iron diamine sequence appeared to impart density to the microfibrillar component of elastic fibers [28].

Gene context of Elastic Tissue

• Accordingly, we propose that fibrillin-1 provides mostly force-bearing structural support, whereas fibrillin-2 predominantly regulates the early process of elastic fiber assembly [29].
• The surfaces of disrupted elastic fibers showed IR for MMP-2 and MMP-9 [30].
• Immunogold labeling of developing nuchal ligament tissue showed that MAGP-2 had regular covalent and periodic (about 56 nm) association with fibrillin-containing microfibrils of elastic fibers in this tissue [31].
• The Pro-regions of lysyl oxidase and lysyl oxidase-like 1 are required for deposition onto elastic fibers [32].
• Our results showed that the pro-regions of LOX and LOXL play a significant role in directing the deposition of both enzymes onto elastic fibers by mediating interactions with tropoelastin [32].

Analytical, diagnostic and therapeutic context of Elastic Tissue

• This localization of emilin in aorta has been confirmed by quantitative evaluation of the distribution of gold particles within elastic fibers [33].
• Electron microscopy revealed that the elastin aggregates are highly unusual in that they contain evenly distributed rod-like filaments, in contrast to the amorphous appearance of normal elastic fibers [34].
• We studied the expression of LTBP-2 in the developing mouse and rat by in situ hybridization, using tropoelastin expression as a marker of tissues participating in elastic fiber formation [16].
• Immunoblotting experiments identified putative LTBP-2 isoforms as a 260-kDa species released into the medium by cultured elastic tissue cells and as larger 290- and 310-kDa species in tissue extracts [35].
• Immunofluorescence studies localized both enzymes to elastin at the earliest stages of elastic fiber assembly [32].

References