Familial Microvillous Atrophy

Ad, Phillips; Schmitz, J

doi:10.1097/00005176-199205000-00003

Cited by 178 publications

(74 citation statements)

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“…Microvilli are anti-invasive as they maintain the structure and absorptive function of the intestinal barrier (48,49). Therefore, all of the infectious enteric microorganisms have interest in effacing microvilli from the surface of the enterocytes.…”

Section: Discussionmentioning

confidence: 99%

Proteolysis of Enteric Cell Villin by Entamoeba histolytica Cysteine Proteinases

Lauwaet

Oliveira

Callewaert

et al. 2003

Journal of Biological Chemistry

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Invasive microorganisms efface enteric microvilli to establish intimate contact with the apical surface of enterocytes. To understand the molecular basis of this effacement in amebic colitis, we seeded Entamoeba histolytica trophozoites on top of differentiated human Caco-2 cell layers. Western blots of detergent lysates from such cocultures showed proteolysis of the actin-bundling protein villin within 1 min of direct contact of living trophozoites with enterocytes. Mixtures of separately prepared lysates excluded detergent colysis as the cause of villin proteolysis. Caspases were not responsible as evidenced by the lack of degradation of specific substrates and the failure of a specific caspase inhibitor to prevent villin proteolysis. A crucial role for amebic cysteine proteinases was shown by prevention of villin proteolysis and associated microvillar alterations through the treatment of trophozoites before coculture with synthetic inhibitors that completely blocked amebic cysteine proteinase activity on zymograms. Moreover, trophozoites of amebic strains pSA8 and SAW760 with strongly reduced cysteine proteinase activity showed a reduced proteolysis of villin in coculture with enteric cells. Salmonella typhimurium and enteropathogenic Escherichia coli disturb microvilli without villin proteolysis, indicating that the latter is not a consequence of the disturbance of microvilli. In conclusion, villin proteolysis is an early event in the molecular cross-talk between enterocytes and amebic trophozoites, causing a disturbance of microvilli.

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Section: Discussionmentioning

confidence: 99%

Proteolysis of Enteric Cell Villin by Entamoeba histolytica Cysteine Proteinases

Lauwaet

Oliveira

Callewaert

et al. 2003

Journal of Biological Chemistry

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“…Gestation is most often unremarkable (5)(6)(7)(8). The diarrhea usually occurs within the first days of life or less often during the second month of life (9). MVID is characterized by a profuse, secretory, watery, severe diarrhea (up to 150 mL Á kg À1 Á day À1 ).…”

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confidence: 99%

Microvillous Atrophy

Perry

Bensallah

Martinez-Vinson

et al. 2014

J. pediatr. gastroenterol. nutr.

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“…MVID is associated with villous atrophy, intracytoplasmic microvillus inclusions, and intracellular accumulation of the normally brush border-enriched metalloendopeptidase CD10 and periodic acid-Schiff (PAS)-positive material in the subapical cytoplasm of intestinal absorptive cells (3,4). Other brush border proteins including sucrase-isomaltase, alkaline phosphatase, sodium-proton exchanger protein 2 and 3 (NHE-2, NHE-3), cyclic guanosine monophosphate-dependent protein kinase, cystic fibrosis transmembrane conductance regulator, and the sodium-glucose transporter 1 have been reported to be present at reduced levels at the apical brush border membrane and to accumulate in the apical cytoplasm (5). At the ultrastructural level, a variable degree of microvillus atrophy, accumulation of secretory granules, and the presence of microvillus inclusions are typically observed.…”

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confidence: 99%