Lipid composition and biophysical properties of membranes in triosephosphate isomerase deficiency

E. Fodor, Tibor Parkas', Susan Hollarr

Research output: Contribution to journalArticle

Abstract

Triosephosphate isomerase (TPI) deficiency is a rare authosomal recessive defect of a glycolytic enzyme causing severe neurolgical disorders and high levels of dihydroxyacetone phosphate in ceils. Erythrocyte membranes collected from members of a TPI deficient hungarian family proved to be more fluid than those from healthy controls as assessed using anthroyloxy fatty acids. The RBC membranes from the propsitus proved to be more fluid than those from his compound hetrozygote brother. Separation by HPI.C of phospholipids into diacyl, alkylacyl, and alkenylacyl subgrups showed a decreased level of ethanolamine plasmalogens without major changes in fatty acid compositions in the lymphocytes but not in erythrocytes or thrombocytes of the propositus. The lymphocytes of the propositus proved to be more rich in certain molecular species (16:0/20:4,18:0/20:4,18:1/20:4) of phopsphatidylethanolamines. Since lymphocytes share a number of structural and functional characteristics with nerve cells it is proposed that the neurological disorders of the propositus may be due to disturbed lipid metabolism of the cellular membranes.

Original languageEnglish
JournalFASEB Journal
Volume11
Issue number9
Publication statusPublished - 1997

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Triose-Phosphate Isomerase
triose-phosphate isomerase
Lymphocytes
lipid composition
erythrocytes
lymphocytes
Membranes
Lipids
Fatty Acids
Chemical analysis
Dihydroxyacetone Phosphate
blood platelets
ethanolamine
Fluids
nervous system diseases
Erythrocyte Membrane
Nervous System Diseases
Lipid Metabolism
lipid metabolism
Neurons

ASJC Scopus subject areas

  • Agricultural and Biological Sciences (miscellaneous)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Biochemistry
  • Cell Biology

Cite this

Lipid composition and biophysical properties of membranes in triosephosphate isomerase deficiency. / Fodor, E.; Parkas', Tibor; Hollarr, Susan.

In: FASEB Journal, Vol. 11, No. 9, 1997.

Research output: Contribution to journalArticle

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