700 VI. OCCURRENCE OF LIPIDS IN THE ANIMAL 



filled cells were not limited to the reticuloendothelial system, but occurred 

 in all organs. Menten and Welton 933 reported the following composition 

 of liver and spleen lipids (based on dry weight), respectively, in the tissues 

 of an infant nine months of age who had died of Niemann-Pick's disease: 

 lecithin, 13.0 and 9.7%; cephalin, 4.1 and 3.4%; crude sphingomyelin, 

 28.0 and 32.1%. Although the sphingomyelin content of all organs was 

 greatly increased, the amount of this phospholipid in the brain remained 

 unchanged. 932 ' 934935 The fatty acids present in the sphingomyelin ob- 

 tained from the different organs in Niemann-Pick's disease were those 

 normally present. 932 - 936 However, in contradistinction to these findings, 

 Klenk 931 reported only stearic acid in the sphingomyelin of diseased brains, 

 while stearic, palmitic, and lignoceric acids occur in the brain sphingo- 

 myelin of normal individuals. However, Tropp and Eckardt 937,938 re- 

 ported that, although stearic acid was found to be the main fatty acid 

 component of the sphingomyelin obtained from the brain, liver, and 

 spleen of patients who had died as a result of Niemann-Pick's disease, some 

 lignoceric and palmitic acids were also found. It is quite possible that, in 

 these latter cases, the disease was not as severe, or had not continued for as 

 long a period, as in the case reported by Klenk. One might well assume 

 that only a partial replacement of lignoceric acid and palmitic acid had been 

 completed when death occurred. However, Thannhauser and Schmidt 922 

 do not consider the experiments indicating the unusual fatty acid com- 

 position of brain sphingomyelin in Niemann-Pick's syndrome to be con- 

 clusive, since the presence of hydrolecithin was not excluded. Sperry 920 

 reported that the level of brain cerebrosides was depressed in this disease. 

 Moreover, Klenk 931 - 936 demonstrated the presence of variable amounts of 

 Substance X, which is a galactoside. 



The sphingomyelin content of the serum of patients suffering from 

 Niemann-Pick's disease has been reported as normal by Thannhauser, 917 by 

 Sperry, 920 and by Chargaff. 939 The latter worker found that the sphingo- 

 myelin content in the serum was 120 milligram per cent, which is within the 

 normal range reported by Thannhauser and Setz 940 as 100 to 180 milligram 

 per cent. However, this finding does not support the hypothesis of 



933 M. L. Menten and J. P. Welton, Am. J. Diseases Children, 72, 720-727 (1946). 



934 H. Sobotka, E. Z. Epstein, and L. Liechtenstein, Arch. Pathol, 10, 677-686 (1930). 



935 E. Epstein and K. Lorenz, Z. physiol. Chem., 211, 217-230 (1932). 



936 E. Klenk, Z. physiol. Chem., 262, 128-143 (1939-1940). 



937 C. Tropp and B. Eckardt, Z. physiol. Chem., 248, 38-42 (1936). 



938 C. Tropp and B. Eckardt, Z. physiol. Chem., 245, 163-167 (1936-1937). 



939 E. Chargaff, «/. Biol. Chem., 130, 503-511 (1939). 



940 S. J. Thannhauser and P. Setz, /. Biol. Chem., 116, 533-541 (1936). 





