Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Spontaneous nephrotic (ICGN) mice develop proteinuria, hypoproteinemia and hypercholesterolemia. These symptoms steadily progress to chronic renal failure. Details of the changes of the kidney, in the late stage (more than 5 months old) were investigated by both light and electron microscopy. The kidney exhibited a slightly whitish, granular surface and the cortex became thinner and contained fibrous lesions, in which clusters of unaffected and occluded renal tubules were randomly scattered. In the juxtamedullary and outer medullary zone, there were highly dilated renal tubules, which sometimes contained urinary casts. The glomerulus exhibited basement membrane thickening in the capillary loops and the capillary lumen was narrowed in size and sometimes occluded. No detachment of the podocyte from the basement membrane was observed and the podocyte foot-processes were extensively fused, causing their characteristic slits to be lost. The thickened basement membranes were found both in the glomerulus and around the occluded renal tubules, while the basement membrane in the dilated renal tubule appeared normal. Therefore, the basement membranes of the glomerulus and renal tubules appear to react differently in the pathogenesis of the condition. In conclusion, ICGN mice are a good model for not only the nephrotic syndrome but also for chronic renal failure.
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PMID:Morphological studies on the kidney of the spontaneous nephrotic (ICGN) mice in the late stage. 778 18

An oxidant stress has been shown to prevail in experimental and clinical nephrotic syndrome. Such oxidant stress may be induced by a reduced activity of antioxidant systems. We examined the altered expression of manganese-superoxide dismutase (Mn-SOD), an antioxidant enzyme, in patients with idiopathic nephrotic syndrome, in whom an increased oxidant stress had been demonstrated. The Mn-SOD activities in peripheral blood mononuclear cells obtained from 12 patients with active nephrotic syndrome (6.0 +/- 1.1 years of age, mean +/- SE) and hypoproteinemia were 42% lower (p < 0.05) than in 12 control subjects (5.5 +/- 0.5 years of age) with normal serum total protein concentrations. Reverse-transcriptase polymerase chain reaction also demonstrated that Mn-SOD messenger RNA expression in the patients with nephrotic syndrome was, on average, 59% lower than in control subjects. Because expressions of some genes are sensitive to serum, the serum dependency of Mn-SOD gene transcription was studied in glomerular endothelial cells transfected with a luciferase reporter gene fused with a rat Mn-SOD DNA fragment of -806 to +22 bp of the transcription initiation site (-806:+22). When these cells were exposed to different concentrations of fetal bovine serum (0.5% to 15%), the transcriptional activities determined by luciferase activities were proportional to serum concentrations. This serum-dependent transcriptional activation was also demonstrated by the fragment (-220:+22) but not by the fragment (-220:-20). When glomerular endothelial cells transfected with the fragment (-220:+22) were treated with 5% serum from patients with active nephrotic syndrome, transcriptional activation was more than 80% less than that by 5% serum from control subjects without nephrosis. These results indicate that Mn-SOD gene transcription is regulated at least in part by serum, and that the serum-dependent transcription of the gene is diminished in patients with idiopathic nephrotic syndrome. The regulatory region of serum-dependent gene transcription resides within its early promoter region. Our findings suggest that down-regulation of antioxidant enzyme transcription may contribute increased oxidant stress in idiopathic nephrotic syndrome.
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PMID:Down-regulation of manganese-superoxide dismutase gene expression in idiopathic nephrotic syndrome. 915 91