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Science  04 May 2001:
Vol. 292, Issue 5518, pp. 815
DOI: 10.1126/science.292.5518.815c

Peroxisome Primer

Although the movie Lorenzo's Oil brought fame to a neurological disorder known as adrenoleukodystrophy, the film didn't spotlight peroxisomes—tiny, fat-metabolizing structures in cells that malfunction in boys with the deadly disease. But now these organelles are getting star billing at the new Peroxisome Web Site at Johns Hopkins University.

Peroxisomes, which are basically bags of enzymes, perform tasks such as breaking down fatty acids, synthesizing cholesterol, and making parts of the myelin sheath that protects nerves. “I wanted a place where scientists could go to quickly find all publications available for a specific gene, or to get background on an aspect of the peroxisome that they are unfamiliar with,” says Katherine Sacksteder, who created the site as part of her Ph.D. thesis with cell biologist Stephen J. Gould. Visitors will find descriptions of the 24 known proteins, or peroxins, involved in the formation and function of these organelles, along with links to PubMed references and gene sequences in GenBank. The site also includes diagrams showing, for instance, bile acid synthesis and the pathways by which proteins are transported into peroxisomes.

Separate sections for lay readers and physicians cover peroxisome-associated disorders such as adrenoleukodystrophy and Zellweger syndrome, a spectrum of typically fatal diseases marked by developmental delays, facial abnormalities, and heart, liver, and kidney dysfunction.

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