The Pathobiochemistry of Gastrointestinal Symptoms in a Patient with Niemann-Pick Type C Disease View Full Text


Ontology type: schema:Chapter      Open Access: True


Chapter Info

DATE

2015-06-30

AUTHORS

Mahdi Amiri , Eva-Maria Kuech , Hadeel Shammas , Gabi Wetzel , Hassan Y. Naim

ABSTRACT

The molecular basis of gastrointestinal intolerances in a severe case of Niemann-Pick type C disease was analyzed in an intestinal biopsy specimen. The enzyme activities of intestinal sucrase-isomaltase and maltase-glucoamylase are reduced in the patient, while that of lactase is comparable to the control. The association of SI with lipid rafts is reduced in the patient’s biopsy as a consequence of altered composition of membrane microdomains. As association with lipid rafts influences the intracellular transport and the enzyme activities of sucrase-isomaltase and maltase-glucoamylase, these data explain reduced carbohydrate digestion in the intestinal lumen and delineate the effect of deficient cholesterol and sphingolipid homeostasis in development of gastrointestinal symptoms in NPC patients. More... »

PAGES

25-29

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/8904_2015_454

DOI

http://dx.doi.org/10.1007/8904_2015_454

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1015823103

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/26122626


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