Stored Cerebroside

as in normals, the double bond of the sphingosine moiety is located between C-4 and C-5 in trans-configuration. 

Cholesterol, free Cholesterol, esterified Total phospholipids Sphingomyelin  

While normally cerebroside sphingosine is present in the erythro-form with regard to the substituents at C-2 (N-acyl residue) and at C-3 (hydroxyl group), the sphingomyelin which is stored in Niemann-Pick disease was found in the threo-form (CuMiNGS 1962). Threo-sphingosine does not occur in GD (Carter et al. 1961). 

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Oberling 1926, Pick 1933). In addition, osteoblasts and fibroblast-like spindle cells of the bone marrow (Block and Jacobson 1948) and adventitial cells and histiocytes store cerebrosides and become GC (Pick 1926). 

Fig. 5. GD. Bono marrow. HE-stain. Enlargement 900-fold. Reticulum cells are transformed to Gaucher cells and store cerebrosides which impart to the cytoplasm a crumbled tissue paper appearance. (Reproduced by courtesy of Prof. P. B. Diezel)...

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