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)

Lysozyme levels were determined in the mucosa of gut in 80 children with chronic inflammatory bowel disease, malabsorption and acrodermatitis enteropathica.l Levels of lysozyme in the mucosa of colon were found to be significantly higher in cases with chronic inflammatory bowel disease, whereas in children with malabsorption (celiac disease) concentration of lysozyme in the mucosa of small intestine were significantly lower compared to a control group. In a 4 months old boy with acrodermatitis enteropathica there was a low level of lysozyme in the mucosa of the small intestine. After therapy with zinc for one year concentration of lysozyme was normalized.
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PMID:[Lysozyme concentrations in the intestinal mucosa in malabsorption syndromes and chronic inflammatory intestinal diseases]. 669 40

Morphological abnormalities in Paneth cells occur in patients with acrodermatitis enteropathica, a hereditary disease associated with zinc deficiency; furthermore, rat Paneth cells contain large amounts of zinc. This study was conducted to assess the effect of severe zinc deficiency in Sprague-Dawley rats on various parameters of Paneth cells. Morphology at both the light microscopical and ultrastructural levels, Paneth cell numbers per crypt and the intracellular distribution of lysozyme were not altered by zinc deficiency. A weak correlation (r = +0.38, P = 0.05) was noted between ileal zinc concentration and numbers of IgA-containing Paneth cells per crypt. These findings indicate that the morphological abnormalities noted in human Paneth cells in patients with acrodermatitis enteropathica cannot be reproduced by experimental severe zinc deficiency in rats. Furthermore, these generally negative findings suggest that the severe diarrhoea often associated with zinc deficiency is not attributable to abnormalities induced in Paneth cells by zinc deficiency.
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PMID:Ileal Paneth cells and IgA system in rats with severe zinc deficiency: an immunohistochemical and morphological study. 744 Feb 49

Mutations in the human Zip4 gene cause acrodermatitis enteropathica, a rare, pseudo-dominant, lethal genetic disorder. We created a tamoxifen-inducible, enterocyte-specific knockout of this gene in mice which mimics this human disorder. We found that the enterocyte Zip4 gene in mice is essential throughout life, and loss-of-function of this gene rapidly leads to wasting and death unless mice are nursed or provided excess dietary zinc. An initial effect of the knockout was the reprogramming of Paneth cells, which contribute to the intestinal stem cell niche in the crypts. Labile zinc in Paneth cells was lost, followed by diminished Sox9 (sex determining region Y-box 9) and lysozyme expression, and accumulation of mucin, which is normally found in goblet cells. This was accompanied by dysplasia of the intestinal crypts and significantly diminished small intestine cell division, and attenuated mTOR1 activity in villus enterocytes, indicative of increased catabolic metabolism, and diminished protein synthesis. This was followed by disorganization of the absorptive epithelium. Elemental analyses of small intestine, liver, and pancreas from Zip4-intestine knockout mice revealed that total zinc was dramatically and rapidly decreased in these organs whereas iron, manganese, and copper slowly accumulated to high levels in the liver as the disease progressed. These studies strongly suggest that wasting and lethality in acrodermatitis enteropathica patients reflects the loss-of-function of the intestine zinc transporter ZIP4, which leads to abnormal Paneth cell gene expression, disruption of the intestinal stem cell niche, and diminished function of the intestinal mucosa. These changes, in turn, cause a switch from anabolic to catabolic metabolism and altered homeostasis of several essential metals, which, if untreated by excess dietary zinc, leads to dramatic weight loss and death.
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PMID:A mouse model of acrodermatitis enteropathica: loss of intestine zinc transporter ZIP4 (Slc39a4) disrupts the stem cell niche and intestine integrity. 2273 83