title: Peroxisomal membrane ghosts in Zellweger syndrome--aberrant organelle assembly.
abstract: Peroxisomes are apparently missing in Zellweger syndrome; nevertheless, some of the integral membrane proteins of the organelle are present. Their distribution was studied by immunofluorescence microscopy. In control fibroblasts, peroxisomes appeared as small dots. In Zellweger fibroblasts, the peroxisomal membrane proteins were located in unusual empty membrane structures of larger size. These results suggest that the primary defect in this disease may be in the mechanism for import of matrix proteins.
authors.affiliation: Rockefeller University, New York, NY 10021.
authors.forename: M J
authors.initials: MJ
authors.lastname: Santos
authors.forename: T
authors.initials: T
authors.lastname: Imanaka
authors.forename: H
authors.initials: H
authors.lastname: Shio
authors.forename: G M
authors.initials: GM
authors.lastname: Small
authors.forename: P B
authors.initials: PB
authors.lastname: Lazarow
mesh_terms: D005347:Fibroblasts / Q000032:analysis / Q000648:ultrastructure; D005455:Fluorescent Antibody Technique; D030342:Genetic Diseases, Inborn / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007425:Intracellular Membranes / Q000032:analysis / Q000473:pathology; D008565:Membrane Proteins / Q000032:analysis*; D008830:Microbodies / Q000032:analysis / Q000473:pathology*; D009940:Organoids / Q000032:analysis / Q000473:pathology; D013577:Syndrome
chemical_list: D008565:Membrane Proteins