Document Detail

Increase of ceramide monohexoside and dipalmitoyl glycerophospholipids in the brain of Zellweger syndrome.
MedLine Citation:
PMID:  17399899     Owner:  NLM     Status:  MEDLINE    
The lipid composition and molecular species of phospholipids were examined in the brain of a patient with Zellweger syndrome (ZS), and were compared with those of control infants. In the cerebral gray matter of the ZS patient, the amounts of ceramide monohexoside and cholesterol ester were larger than those of controls. By contrast, the amount of ceramide monohexoside in the white matter was smaller in the ZS patient than that in the age-matched control. Although the amount of phosphatidylcholine (PC) plus phosphatidylserine (PS) was the same, dipalmitoyl PC and PS were increased in both the gray and white matter of the ZS cerebrum. These alterations in the molecular species of brain lipids may play crucial roles in the pathogenesis of ZS.
Makiko Saitoh; Yoichi Sakakihara; Masashi Mizuguchi; Masayuki Itoh; Sachio Takashima; Masao Iwamori; Shigehiko Kamoshita; Takashi Igarashi
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2007-03-15
Journal Detail:
Title:  Neuroscience letters     Volume:  417     ISSN:  0304-3940     ISO Abbreviation:  Neurosci. Lett.     Publication Date:  2007 May 
Date Detail:
Created Date:  2007-04-16     Completed Date:  2007-07-25     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7600130     Medline TA:  Neurosci Lett     Country:  Ireland    
Other Details:
Languages:  eng     Pagination:  165-70     Citation Subset:  IM    
Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Japan.
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MeSH Terms
1,2-Dipalmitoylphosphatidylcholine / metabolism*
Cerebral Cortex / metabolism*,  physiopathology
Cerebrosides / metabolism*
Lipid Metabolism / genetics*
Membrane Lipids / genetics,  metabolism*
Nerve Degeneration / genetics,  metabolism,  physiopathology
Nerve Fibers, Myelinated / metabolism,  pathology
Neurons / metabolism,  pathology
Phosphatidylserines / metabolism
Up-Regulation / genetics
Zellweger Syndrome / metabolism*,  physiopathology
Reg. No./Substance:
0/Cerebrosides; 0/Membrane Lipids; 0/Phosphatidylserines; 0/ceramide monohexoside; 2644-64-6/1,2-Dipalmitoylphosphatidylcholine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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