No abstract

The authors study the influence of bromhexine metabolite VIII on phosphohpid and fatty acid composition comparatively to controls, The subcutaneous injection of NA 872 produces on pulmonary surfactant an increase of 43% of total phospholipids. This change in the distribution of phospholipids is shown by the increase of 53% of phosphatidylcholines. A rise of 61.9–74.4% of palmitic acid appears in the phosphatidylcholines of the pulmonary surfactant. It can be noticed that the specific activity of the phosphatidylcholines rises by 31%.

No abstract

Lymphoid cell lines (LCL) from 3 adult patients with non-neuropathic Gaucher disease were established by Epstein-Barr virus (EBV) transformation and were investigated from the view of enzymology. (1) Glucosylceramide-ß-glucosidase (GlcCer-ß-glucosidase) was present in soluble and particulate fraction of LCL from normal subjects and was deficient in type 1 Gaucher LCL; the deficiency of all molecular forms, shown by electrofocusing, indicates that they are coded by the same gene. (2) The existence of two non-specific ßglucosidases, one soluble (minor), the other membrane-bound (major), was demonstrated in leucocytes and LCL from normals; in Gaucher LCL, these were also present in a normal range. (3) Characteristic properties of the non-specific membrane-bound ß-glucosidase were defined: lability at acidic pH and strong inhibitory effect by detergents. These properties allowed to discriminate it from the lysosomal GlcCer-ß-glucosidase and to define optimal assay conditions for determination of residual GlcCer-ß-glucosidase activity in Gaucher disease, using artificial substrate, without interference of non-specific membrane-bound ß-glucosidase. These results demonstrate that EBV-transformed LCL represent an accurate model system for enzymatic studies of Gaucher disease.

No abstract