LIPID STORAGE UNDER ABNORMAL CONDITIONS 703 



described a variation in an infant ; in this case the Gaucher cells were also 

 found in the pulmonary and cerebral tissue, lymphatic glands, liver, bone- 

 marrow, and thymus. 



Pick 919 postulated that Gaucher's disease is a storage disease analogous 

 to xanthomatosis. It was believed that an extra amount of cerasine was 

 supplied in the blood stream to the several organs which were able to re- 

 move it and accumulate it. However, this hypothesis seems unlikely, 

 since cerasine and other cerebrosides are not normal components of the 

 blood, 956 - 957 and have not been demonstrated in the serum in Gaucher's 

 disease. 957958 Although Erickson et a/. 959 reported that cerebrosides are 

 present in normal serum, their methods were based upon indirect analyses 

 and did not involve isolation of the cerebrosides. Thannhauser and 

 Schmidt 922 are convinced that cerebrosides do not occur in normal blood, or 

 in that of patients suffering from Gaucher's disease. However, when highly 

 purified cerebrosides were injected intraperitoneally into rabbits, 960 morpho- 

 logic changes resembling those of Gaucher's disease in man were found, al- 

 though they were less extensive. Moreover, increased levels of cerebro- 

 sides were observed in the liver, spleen, and lymph-nodes of the rabbits, 

 even after the oral administration of cerebrosides. 



On the other hand, Thannhauser 917 suggested that the cerebrosides 

 originate in the same cells in which they are found. This would imply an 

 overproduction, and a resultant accumulation of this metabolite in the 

 cells of the several organs in which the cerebrosides are concentrated. 

 This condition might arise from a disturbance of the intracellular enzymes 

 concerned with either the anabolism or the catabolism of the cerebrosides. 

 This explanation is analogous to that proposed for the etiology of essential 

 xanthomatosis of the normocholesteremic type, and for that of Niemann- 

 Pick's disease. 



Another finding which has been interpreted as evidence against the trans- 

 port theory accounting for the presence of an excess amount of cerebrosides 

 in the spleen in Gaucher's disease is the discovery that the cerebrosides in 

 the latter organ may differ from those in the brain. Thus, Halliday et 



966 S. J. Thannhauser, J. Benotti, and H. Reinstein, /. Biol. Chem., 129, 709-716 

 (1939). 



957 J. Bruckner, Z. physiol. Chem., 268, 251-256 (1941). 



958 E. Dworacek and H. Pesta, Wien. klin. Wochschr., 52, 332-337 (1939). 



969 B. N. Erickson, H. J. Souders, M. L. Shepherd, D. M. Teague, and H. H. Williams, 

 Proc. Soc. Exptl. Biol. Med., 45, 153-156 (1940). 



963 O. O. Christianson, Arch. Pathol, 32, 369-377 (1941). 



