Quantitative Chromatographic Analysis of the Phospholipids of Abnormal Human Red Blood Cells.

Studies of Red-Cell Stromal Lipids in Tay-Sachs Disease and Other Lipidoses*

“…The results obtained in the second 10 to 15%o was in this category ( Figure 1), a reextract are presented in Tables IV and VI. sult that agrees well with the data of Weed, Reed, and Berg (27). The small peaks of phosphoruscontaining compounds eluted before the cephalin peak (A) in the system used in this study and possibly containing cephalin plasmalogens may have been included in the cephalins in the experiments just cited (24)(25)(26) and may account for the differences observed. Farquahar (26) (26,27,29).…”

“…Published studies of red-cell phospholipids in various other diseases have shown no comparable abnormalities. Thus, Phillips and Roome (25) showed that red-cell phospholipids are normal in a variety of hemolytic disorders, whereas Ways, Reed, and Hanahan (32) and Phillips (29) have reported increased sphingomyelin and decreased lecithin concentrations in the red cells of patients with acanthocytosis. There may be at least two traits involved in the inheritance of Tay-Sachs, Niemann-Pick, and Gaucher's diseases, one of which is common to all three diseases and which determines the changes we have observed in the red cells, whereas the other (s) determines the specific sphingolipid accumulating in the nervous system or the reticuloendothelial system, or the predisposition of these tissues for such accumulation.…”

The role of membrane lipids in the survival of red cells in hereditary spherocytosis

“…There was no accompanying difference in the serum concentrations of either cholesterol (pa- (Table I). The lipid content of these cells was similar to that of red cells from 11 normal subjects with intact spleens (Table I), as previously reported (5,13,14). However, when compared with red cells from patients whose spleens had been removed for reasons other than HS, the HS cells had a deficiency of cholesterol that averaged 19.3% and of phospholipid that averaged 15.7%.…”

“…Because previous studies had compared red cell lipids from patients with HS who lacked spleens with those of normal subjects with spleens (5,13,14), the effect of splenectomy, per se, was evaluated. Measurements were made of the lipid content of red cells from 14 patients who had undergone splenectomy for reasons other than HS (Table I).…”

“…In patients with spleens, the lipid content of HS red cells is less than normal (5); however, after splenectomy red cell lipids in HS are the same as those in red cells from normal subjects with intact spleens (5,13,14). Thus, whereas depletion of membrane lipid may explain, in part, the events of splenic conditioning and eventual cell destruction, the fact that after splenectomy HS red cells have a lipid content that is "normal" has excluded a quantitative abnormality in red cell lipids from consideration as the basic defect in HS.…”