Increase of ceramide monohexoside and dipalmitoyl glycerophospholipids in the brain of Zellweger syndrome.

Abstract

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.

• Authors: Makiko Saitoh, Yoichi Sakakihara, Masashi Mizuguchi, Masayuki Itoh, Sachio Takashima, Masao Iwamori, Shigehiko Kamoshita, Takashi Igarashi
• Journal: Neuroscience letters (Neurosci Lett) Vol. 417 Issue 2 Pg. 165-70 (May 01 2007) ISSN: 0304-3940 [Print] Ireland
• PMID: 17399899 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Cerebrosides
• Membrane Lipids
• Phosphatidylserines
• ceramide monohexoside
• 1,2-Dipalmitoylphosphatidylcholine

Topics

• 1,2-Dipalmitoylphosphatidylcholine (metabolism)
• Cerebral Cortex (metabolism, physiopathology)
• Cerebrosides (metabolism)
• Humans
• Infant
• Lipid Metabolism (genetics)
• Male
• 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)