title: Membrane fatty acids, glutathione-peroxidase activity, and cation transport systems of erythrocytes and malondialdehyde production by platelets in Laurence Moon Barter Biedl syndrome.
abstract: The fatty acid composition of erythrocyte membrane, the glutathione-peroxidase activity of erythrocytes and platelets, the production of malondialdehyde by platelets and the activity of the main systems of transmembrane cation transport have been studied in 5 members of a family, 2 of whom affected by Laurence-Moon-Barter-Biedl Syndrome. A remarkable increase of polyunsaturated fatty acids (particularly arachidonic acid) and of cholesterol/phospholipid molar ratio has been noted. This pattern of membrane lipids was associated to an increment of malondialdehyde production and an increase activity of glutathione-peroxidase. Serum retinol and a-tocopherol were in the normal range, whereas serum selenium was low in 3 out of 5 members. Moreover, the alteration of membrane lipids was associated to a decrease of the maximal velocity of Li-Na countertransport. We speculate that the enrichment of polyunsaturated fatty acids on the cell membranes may represent a condition favoring the lipoperoxidation and therefore the development of the retinitis pigmentosa characteristic feature of Laurence-Moon-Barter-Biedl Syndrome.
authors: Istituto di Patologia Medica, Università di Verona, Italy.
authors: R
authors: R
authors: Corrocher
authors: L
authors: L
authors: Guadagnin
authors: M
authors: M
authors: de Gironcoli
authors: D
authors: D
authors: Girelli
authors: P
authors: P
authors: Guarini
authors: O
authors: O
authors: Olivieri
authors: S
authors: S
authors: Caffi
authors: A M
authors: AM
authors: Stanzial
authors: S
authors: S
authors: Ferrari
authors: L
authors: L
authors: Grigolini
mesh_terms: D000293:Adolescent; D000328:Adult; D001693:Biological Transport, Active; D001792:Blood Platelets / Q000378:metabolism*; D004910:Erythrocyte Membrane / Q000378:metabolism; D004912:Erythrocytes / Q000378:metabolism*; D005260:Female; D005979:Glutathione Peroxidase / Q000032:analysis*; D006801:Humans; D007849:Laurence-Moon Syndrome / Q000097:blood*; D008297:Male; D008314:Malonates / Q000378:metabolism*; D008315:Malondialdehyde / Q000378:metabolism*; D008563:Membrane Lipids / Q000378:metabolism; D008875:Middle Aged; D012174:Retinitis Pigmentosa / Q000209:etiology; D012643:Selenium / Q000097:blood; D012964:Sodium / Q000378:metabolism; D014801:Vitamin A / Q000097:blood; D014810:Vitamin E / Q000097:blood
chemical_list: D008314:Malonates; D008563:Membrane Lipids; D014801:Vitamin A; D014810:Vitamin E; D008315:Malondialdehyde; D012964:Sodium; D005979:Glutathione Peroxidase; D012643:Selenium