Human Heredity
Original Paper
Complementation in Zellweger Syndrome: Biochemical Analysis of Newly Generated PeroxisomesStanczak H.a · Kremser K.a · Singh A.K.b,c · Ashcraft J.a · Stanley W.d · Singh I.aDepartments of aPediatrics and bPathology, Medical University of South Carolina, Charleston, S.C., USA; cDepartment of Pathology and Laboratory Medicine, Veterans Affairs Medical Center, Charleston, S.C., USA; dChildren’s National Medical Center and Department of Pediatrics, and Pathology, George Washington University, Washington, D.C., USA
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Article / Publication Details
Published online: September 02, 2008
Issue release date: 1992
Number of Print Pages: 7
Number of Figures: 0
Number of Tables: 0
ISSN: 0001-5652 (Print)
eISSN: 1423-0062 (Online)
For additional information: https://www.karger.com/HHE
Abstract
The Zellweger syndrome is characterized by a defect which results in the abnormal biogenesis of peroxisomes. As a consequence, metabolic activities associated with peroxisomes such as the oxidation of very long chain fatty acids, the synthesis of plasmalogens, and the catabolism of phytanic and pipecolic acids are impaired. Since this disorder is genetically heterogeneous and several complementation groups are known, we were able to study the normalization of peroxisomal activity during the process of complementation. The restoration of catalase and dihydroxyacetone phosphate acyltransferase activities peaked within 3-4 days postfusion while the oxidation of lignoceric acid was much delayed (7-8 days). Electron microscopy indicated that by 6 days following hybridization, peroxisome structure and density in heterokaryons was comparable to normal control cells. The heterogenous biochemical response during peroxisome normalization could be due to several factors including a possible requirement for restoration of peroxisomal structural integrity for maximum activation of certain metabolic pathways.
© 1992 S. Karger AG, Basel
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Article / Publication Details
Published online: September 02, 2008
Issue release date: 1992
Number of Print Pages: 7
Number of Figures: 0
Number of Tables: 0
ISSN: 0001-5652 (Print)
eISSN: 1423-0062 (Online)
For additional information: https://www.karger.com/HHE
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