Biology:Glucocerebroside
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Short description: Family of lipids
Glucocerebroside (also called glucosylceramide) is any of the cerebrosides in which the monosaccharide head group is glucose.
Clinical significance
In Gaucher's disease, the enzyme glucocerebrosidase is nonfunctional and cannot break down glucocerebroside into glucose and ceramide in the lysosome.[1] Affected macrophages, called Gaucher cells, have a distinct appearance similar to "wrinkled tissue paper" under light microscopy, because the substrates build-up within the lysosome. [2]
See also
- Glucosylceramide synthase
- Gaucher's disease
- Glucocerebrosidase
References
- ↑ "A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments". International Journal of Molecular Sciences 18 (2): 441. Feb 2017. doi:10.3390/ijms18020441. PMID 28218669.
- ↑ "Gaucher Disease: The Metabolic Defect, Pathophysiology, Phenotypes And Natural History". Pediatr Endocrinol Rev 12 (1): 72–81. Sep 2014. PMID 25345088.
External links
- Glucocerebrosides at the US National Library of Medicine Medical Subject Headings (MeSH)
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