Glucocerebroside Explained

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

Notes and References

1. 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 . A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments . International Journal of Molecular Sciences. 18 . 2 . 441 . Feb 2017 . 28218669 . 10.3390/ijms18020441. 5343975 . free .
2. Baris HN, Cohen IJ, Mistry PK . Gaucher Disease: The Metabolic Defect, Pathophysiology, Phenotypes And Natural History . Pediatr Endocrinol Rev . 12 . 1 . 72–81 . Sep 2014 . 25345088. 4520262 .