Publication Date:
1988-03-25
Description:
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.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Santos, M J -- Imanaka, T -- Shio, H -- Small, G M -- Lazarow, P B -- AM19394/AM/NIADDK NIH HHS/ -- New York, N.Y. -- Science. 1988 Mar 25;239(4847):1536-8.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Rockefeller University, New York, NY 10021.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/3281254" target="_blank"〉PubMed〈/a〉
Keywords:
Fibroblasts/analysis/ultrastructure
;
Fluorescent Antibody Technique
;
Genetic Diseases, Inborn/metabolism/*pathology
;
Humans
;
Intracellular Membranes/analysis/pathology
;
Membrane Proteins/*analysis
;
Microbodies/analysis/*pathology
;
Organoids/analysis/pathology
;
Syndrome
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
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