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The Gaucher Molecule

JAMA. 1969;207(9):1707. doi:10.1001/jama.1969.03150220123020.
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The striking histologic changes in the disease of the spleen described by Gaucher in 1882 captured the curiosity of the pathologist from the first. Despite the improbability that any one physician will ever see a case, he will know something about Gaucher's disease—enough that mention of the lipid content of the characteristic cells will provoke the yawnings of boredom.

Even electron microscope identification of the numerous slender tubules within a cytoplasmic sac is "old hat." If one attempts a conversation piece with, "As you know, the sphingosine-glucose-cerebrosides probably have alternating hydrophobic and hydrophilic portions," his listeners will doze off at sphingosine and be fast asleep with cerebroside.

Nevertheless, the case is not quite closed, although Robert E. Lee, MD,1 has painted a portrait of the molecule (not in the tempera of Rembrandt, to be sure), but showing that the Gaucher tubules are composed of fibrils arranged in a "gentle

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