(A) Right eye of a 28-month-old boy before topical cysteamine therapy, showing abundant crystals. (B) Same eye as in A after 7 months of 0.5 percent cysteamine.

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Metabolism of ganglioside GM2 in GM2 activator-deficient fibroblasts

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(A) Right eye of a 28-month-old boy before topical cysteamine therapy, showing abundant crystals. (B) Same eye as in A after 7 months of 0.5 percent cysteamine eyedrops administered 8 to 12 times per day, with clearing of corneal crystals. (C) Left eye of 21-year-old woman prior to cysteamine eyedrop therapy. Uniform haziness typifies appearance of cornea at this age. (D) Same eye as in C after several months of diligent therapy with 0.5 percent cysteamine eyedrops. Cornea is clear to inspection, although crystals remain visible on slit-lamp examination. (Courtesy of Dr. MI Kaiser-Kupfer, National Eye Institute, National Institutes of Health, Bethesda, Maryland.) Source: Cystinosis: A Disorder of Lysosomal Membrane Transport, The Online Metabolic and Molecular Bases of Inherited Disease Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular Bases of Inherited Disease; 2014 Available at: https://ommbid.mhmedical.com/DownloadImage.aspx?image=/data/books/971/ch199fg8.png&sec=62655418&BookID=971&ChapterSecID=62655259&imagename= Accessed: October 02, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved