Increase of ceramide monohexoside and dipalmitoyl glycerophospholipids in the brain of Zellweger syndrome.
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.
Saitoh
Makiko
M
Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Japan. makisaito-tky@umin.ac.jp
Sakakihara
Yoichi
Y
Mizuguchi
Masashi
M
Itoh
Masayuki
M
Takashima
Sachio
S
Iwamori
Masao
M
Kamoshita
Shigehiko
S
Igarashi
Takashi
T
D015060:1,2-Dipalmitoylphosphatidylcholine / Q000378:metabolism*; D002540:Cerebral Cortex / Q000378:metabolism* / Q000503:physiopathology; D002554:Cerebrosides / Q000378:metabolism*; D006801:Humans; D007223:Infant; D050356:Lipid Metabolism / Q000235:genetics*; D008297:Male; D008563:Membrane Lipids / Q000235:genetics / Q000378:metabolism*; D009410:Nerve Degeneration / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D009413:Nerve Fibers, Myelinated / Q000378:metabolism / Q000473:pathology; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D010718:Phosphatidylserines / Q000378:metabolism; D015854:Up-Regulation / Q000235:genetics; D015211:Zellweger Syndrome / Q000378:metabolism* / Q000503:physiopathology
D002554:Cerebrosides; D008563:Membrane Lipids; D010718:Phosphatidylserines; C013870:ceramide monohexoside; D015060:1,2-Dipalmitoylphosphatidylcholine