Glucocerebroside
Glucocerebroside(also calledglucosylceramide) is any of thecerebrosidesin which themonosaccharidehead group isglucose.
Clinical significance
[edit]InGaucher's disease,the enzymeglucocerebrosidaseis nonfunctional and cannot break down glucocerebroside intoglucoseandceramidein the lysosome.[1]Affectedmacrophages,called Gaucher cells, have a distinct appearance similar to "wrinkled tissue paper" underlight microscopy,because the substrates build-up within the lysosome.[2]
See also
[edit]References
[edit]- ^Stirnemann J, Belmatoug N, Camou F, Serratrice C, Froissart R, Caillaud C, Levade T, Astudillo L, Serratrice J, Brassier A, Rose C, de Villemeur TB, Berger MG (Feb 2017)."A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments".International Journal of Molecular Sciences.18(2): 441.doi:10.3390/ijms18020441.PMC5343975.PMID28218669.
- ^Baris HN, Cohen IJ, Mistry PK (Sep 2014)."Gaucher Disease: The Metabolic Defect, Pathophysiology, Phenotypes And Natural History".Pediatr Endocrinol Rev.12(1): 72–81.PMC4520262.PMID25345088.
External links
[edit]
Media related toGlucocerebrosideat Wikimedia Commons- Glucocerebrosidesat the U.S. National Library of MedicineMedical Subject Headings(MeSH)
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