Document Detail

Complementation in Zellweger syndrome: biochemical analysis of newly generated peroxisomes.
MedLine Citation:
PMID:  1511996     Owner:  NLM     Status:  MEDLINE    
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.
H Stanczak; K Kremser; A K Singh; J Ashcraft; W Stanley; I Singh
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Human heredity     Volume:  42     ISSN:  0001-5652     ISO Abbreviation:  Hum. Hered.     Publication Date:  1992  
Date Detail:
Created Date:  1992-09-28     Completed Date:  1992-09-28     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0200525     Medline TA:  Hum Hered     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  172-8     Citation Subset:  IM    
Department of Pediatrics, Medical University of South Carolina, Charleston 29425.
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MeSH Terms
Acyltransferases / metabolism
Cell Line
Fatty Acids / metabolism
Genetic Complementation Test
Microbodies / metabolism*,  ultrastructure
Zellweger Syndrome / genetics*,  metabolism,  pathology
Grant Support
Reg. No./Substance:
0/Fatty Acids; 557-59-5/lignoceric acid; EC 2.3.-/Acyltransferases; EC O-acyltransferase

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