Changes in the amounts of myelin lipids and molecular species of plasmalogen PE in the brain of an autopsy case with d-bifunctional protein deficiency.

Changes in the amounts of myelin lipids and molecular species of plasmalogen PE in the brain of an autopsy case with d-bifunctional protein deficiency. Research Abstract Details 

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Changes in the amounts of myelin lipids and molecular species of plasmalogen PE in the brain of an autopsy case with d-bifunctional protein deficiency. Abstract Text:

Changes in the molecular species of lipids associated with peroxisomal d-bifunctional protein (d-BP) deficiency were investigated in cerebral tissues to elucidate the pathological mechanisms underlying this disorder. Total phospholipids in the gray and white matters of the patient's brain were decreased to approximately 73% and 50% of control levels, respectively, and profound declines in myelin lipids, i.e. galactosyl ceramide and sulfatides, indicated dysmyelination in our patient with d-BP deficiency. Although the total ganglioside amounts in the gray and white matter of this patient's brain were also decreased to 61% and 37% of control levels and GM1 in the white matter was 20% of the control level, the relative amounts of GM2 in both the gray and the white matter of this patient's brain were increased in comparison to those in the control, indicating altered metabolism of gangliosides. In addition, among molecular species of phospholipids, plasmalogen-type and polyunsaturated fatty acid-containing phosphatidylethanolamine were characteristically decreased in the patient's gray matter. These alterations in the molecular species of brain lipids may affect sensitivity to oxidative stress and the membrane fluidity of neural cells, thereby producing the brain pathology of d-BP deficiency.

Changes in the amounts of myelin lipids and molecular species of plasmalogen PE in the brain of an autopsy case with d-bifunctional protein deficiency. Publishing Authors By Initials

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Changes in the amounts of myelin lipids and molecular species of plasmalogen PE in the brain of an autopsy case with d-bifunctional protein deficiency. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Neuroscience letters

VOLUME: 442

Page Numbers: 4-9

Journal Abbreviation: Neurosci. Lett.

ISSN: 0304-3940

DAY: 1

MONTH: 07

YEAR: 2008

Changes in the amounts of myelin lipids and molecular species of plasmalogen PE in the brain of an autopsy case with d-bifunctional protein deficiency. Information

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LANGUAGE: eng

NlmUniqueID: 7600130

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Grant and Affiliation Information for Changes in the amounts of myelin lipids and molecular species of plasmalogen PE in the brain of an autopsy case with d-bifunctional protein deficiency.

AFFILIATION: Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-ku, Tokyo 113-0033, Japan.

Country: Ireland

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MEDLINETA: Neurosci Lett

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