Peroxisomal Membrane Ghosts in Zellweger Syndrome--Aberrant Organelle Assembly

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 pero...

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Bibliographic Details
Published in:Science (American Association for the Advancement of Science) 1988-03, Vol.239 (4847), p.1536-1538
Main Authors: Santos, M. J., Imanaka, T., Shio, H., Small, G. M., Lazarow, P. B.
Format: Article
Language:English
Subjects:
Antibodies
Antiserum
Biochemistry
Biological and medical sciences
Disease
Errors of metabolism
Fibroblasts
Fibroblasts - analysis
Fibroblasts - ultrastructure
Fluorescence
Fluorescence microscopy
Fluorescent Antibody Technique
Fluorescent antibody techniques
Genetic Diseases, Inborn - metabolism
Genetic Diseases, Inborn - pathology
Humans
Intracellular Membranes - analysis
Intracellular Membranes - pathology
Lipids (lysosomal enzyme disorders, storage diseases)
Liver
Lysosomes
man
Medical research
Medical sciences
Membrane proteins
Membrane Proteins - analysis
Metabolic diseases
Microbodies
Microbodies - analysis
Microbodies - pathology
Organelles
Organoids - analysis
Organoids - pathology
Peroxisomes
Syndrome
Zellweger syndrome
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Description
Summary: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.
ISSN:0036-8075
1095-9203
DOI:10.1126/science.3281254