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)

Multilocular renal cyst is an uncommon lesion of uncertain pathogenesis seen in children and adults. We report the immunohistochemical and lectin-binding profiles of three MRC occurring in adults. All cases had strong and uniform cytoplasmic staining of lining epithelial cells for keratin and binding sites for arachis hypogaea lectin, similar to that seen for the distal convoluted tubules or collecting ducts in normal kidney. However, we found variable expression of other distal nephron markers, including epithelial membrane antigen and Ber-EP4. Furthermore, lining cells in some lesions coexpressed proximal nephron markers such as alpha-1-antitrypsin and lysozyme, as well as binding sites for lotus tetragonolobus lectin. Immunostaining for type IV (basement membrane) collagen demonstrated a continuous subepithelial basement membrane zone and basal laminae surrounding desmin-positive stromal cells. Areas of active collagen synthesis and stromal procollagen deposition were visualized within the interlocular septae using a monoclonal antibody to type I procollagen. Significant proliferative activity was not detected in the lining epithelium or stroma using the anti-proliferating cell nuclear antigen. In conclusion, MRC show aberrant tubular epithelial glycoprotein and glycoconjugate expression, low proliferative activity, and associated activation of interlocular stromal cells.
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PMID:Multilocular renal cyst. Immunohistochemical and lectin-binding study. 137 21

We describe the morphology and comparative genomic hybridization findings in a tumor for which we propose the term "spiradenocylindroma" of the kidney. The tumor arose in the wall of a renal cyst in an otherwise healthy male patient who had a favorable clinical course after nephrectomy. Tumor cells formed either large nodules exhibiting a solid or trabecular architecture with conspicuous perivascular spaces or cylindromatous small tumor cell islands arranged in a jigsaw pattern. Focally, there were interspersed tubular structures and tumor cell rosettes with central deposits of periodic acid-Schiff-positive material. A minor tumor component showed epidermoid differentiation. The tumor cells were strongly positive for cytokeratins 5/6, high molecular weight cytokeratins 34betaE12 and AE1/3, and E-cadherin, but only weakly positive for cytokeratins 7, 8, 18, 19, and epithelial membrane antigen. Focal reactivity for actin, vimentin, and S-100 protein or lysozyme and alpha 1 -antichymotrypsin within tubular and cylindromatous areas suggested myoepithelial and apocrine differentiation, respectively. By comparative genomic hybridization, the only abnormality was loss of the long arm of chromosome 16 and gain of genetic material on the short arm of chromosome 16, suggesting isochromosome i(16p). This finding is unique among renal neoplasms and implies loss of heterozygosity at 16q12-13 of the CYLD1 gene that is critically involved in the oncogenesis of familial cylindromatosis and some sporadic spiradenocylindromas. We conclude that somatic mutation of the CYLD1 gene outside the skin can have a role in the oncogenesis of tumors with cylindromatous features.
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PMID:Spiradenocylindroma of the kidney: clinical and genetic findings suggesting a role of somatic mutation of the CYLD1 gene in the oncogenesis of an unusual renal neoplasm. 1175 79