Human Molecular Genetics, Vol 4, 1791-1798, Copyright © 1995 by Oxford University Press
N Braverman, G Dodt, SJ Gould and D Valle
The peroxisome is a ubiquitous, subcellular organelle containing more than
50 matrix enzymes that participate in a diverse array of metabolic
pathways. Failure to assemble normal peroxisomes is the cellular hallmark
of Zellweger syndrome and other human disorders of peroxisome biogenesis.
Identification of the genes required for peroxisome biogenesis is
proceeding at a rapid pace helped immeasurably by work in other species,
particularly various yeasts. The ultimate goals of this effort are to
identify all of these genes and to understand how their protein products
interact to produce normal appearing and functioning peroxisomes.
Attainment of these goals will lead to a better understanding of the
peroxisome biogenesis disorders, their pathophysiology and treatment.
REVIEWS
Disorders of peroxisome biogenesis
Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
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