Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.2.1.17 (lysozyme)
 21,489 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Elastofibroma is a rare lesion characterized by the presence of abundant abnormal elastic fibers with a unique morphology, fibroblastic proliferation, and collagen deposition. Whether the altered morphology of the elastic fibers is a degenerative phenomenon or is due to abnormal elastogenesis is controversial. We studied fetal skin and three cases of elastofibroma by light microscopy and immunohistochemistry using an antibody to lysozyme, and one case of elastofibroma by electron microscopy (EM). Our previous studies have shown that normal elastic fibers in adult skin do not stain for lysozyme whereas abnormal elastic fibers in solar elastosis and pseudoxanthoma elasticum react positively for lysozyme. In the fetal skin and all three cases of elastofibroma the elastic fibers were negative for lysozyme. EM showed the abnormal flowerlike configuration of the elastic fibers, which consisted of a central core of normal or degenerating elastin surrounded by radiating spokes of granular and filamentous material of variable electron densities, suggesting that the structure and organization of the microfibrils is abnormal. The absence of lysozyme in the aberrant elastic did not differentiate whether there was excessive production of fetal or adult elastic. However, the excessive amount of microfibrils seen at the ultrastructural level suggests that there may be excessive fetal elastic production. The elastic fibers were intimately related to the fibroblasts and were often present within their caveolae, suggesting that the abnormal elastic fibers are produced by the fibroblast. Our study suggests that abnormal elastogenesis with subsequent degeneration plays a role in the production of the abnormal elastic fibers in elastofibroma.
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PMID:"Aberrant elastic" in elastofibroma: an immunohistochemical and ultrastructural study. 777 Sep 61

Elastofibroma is a rare fibrous lesion that most commonly occurs in periscapular soft tissues and is characterized by accumulated abnormal elastic fibers. Although the lesion is generally regarded as a reactive process, an unusual fibroblastic pseudotumor, or as a fibroelastic tumor-like lesion, its etiology remains largely unknown. Recent cytogenetic demonstrations of chromosomal instability and some recurrent or clonal chromosomal changes have raised the possibility that the lesion represents a neoplastic process. We analyzed 14 cases of elastofibroma to further explore, morphologically and genetically, the characteristics of its cellular composition. The interspersed spindle or stellate cells showed a fibroblast-like appearance and were almost consistently positive for vimentin and frequently positive for CD34 and lysozyme immunohistochemically. No spindle cells of myofibroblastic phenotype were recognized. To assess the clonality of the lesions in female patients, the X-linked polymorphic human androgen receptor gene assay was performed using formalin-fixed, paraffin-embedded tissues. A nonrandom inactivation of the androgen receptor gene was detected in two informative cases. Thus, these findings suggest that CD34-positive mesenchymal cells are an integral component of elastofibroma, which represents a clonal fibrous proliferation.
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PMID:Elastofibroma: clonal fibrous proliferation with predominant CD34-positive cells. 1613 60