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© The Rockefeller University Press, 0021-9525/1999//255 $5.00
The Journal of Cell Biology, Volume 144, Number 2, , 1999 255-266

Regular Articles

Peroxisome Synthesis in the Absence of Preexisting Peroxisomes



Sarah T. South and Stephen J. Gould

Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205

Zellweger syndrome and related diseases are caused by defective import of peroxisomal matrix proteins. In all previously reported Zellweger syndrome cell lines the defect could be assigned to the matrix protein import pathway since peroxisome membranes were present, and import of integral peroxisomal membrane proteins was normal. However, we report here a Zellweger syndrome patient (PBD061) with an unusual cellular phenotype, an inability to import peroxisomal membrane proteins. We also identified human PEX16, a novel integral peroxisomal membrane protein, and found that PBD061 had inactivating mutations in the PEX16 gene. Previous studies have suggested that peroxisomes arise from preexisting peroxisomes but we find that expression of PEX16 restores the formation of new peroxisomes in PBD061 cells. Peroxisome synthesis and peroxisomal membrane protein import could be detected within 2–3 h of PEX16 injection and was followed by matrix protein import. These results demonstrate that peroxisomes do not necessarily arise from division of preexisting peroxisomes. We propose that peroxisomes may form by either of two pathways: one that involves PEX11-mediated division of preexisting peroxisomes, and another that involves PEX16-mediated formation of peroxisomes in the absence of preexisting peroxisomes.

Key Words: membrane biogenesis • Zellweger syndrome • peroxisomal membrane protein import • PEX16 • peroxisome biogenesis disorders

Abbreviations used in this paper: DPBS, Dulbecco's phosphate-buffered saline; PBD, peroxisome biogenesis disorders; PMP, peroxisomal membrane protein.

Address correspondence to Dr. Stephen J. Gould, The Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, MD 21205. Tel.: (410) 955-3085 ext. 3424. Fax: (410) 955-0215. E-mail: Stephen.Gould{at}qmail.bs.jhu.edu


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