Lipid and lipoprotein metabolism was studied in patients with recessive X-linked ichthyosis, a disease in which cholesterol sulfatase activity was absent.
1. Polyacrylamide disc-gel electrophoretic mobility of plasma LDL in these patients was greatly increased, as compared with control subjects. This finding could be caused by strong electronegativity of an accumulated cholesterol sulfate containing in plasma LDL.
2. Lipid and protein compositions of VLDL, LDL, HDL2 and HDL3 separated ultracentrifugally in these patients were similar to those of control subjects. In these patients, no abnormal change of serum Apo B level was found. Although serum Apo A level was slightly low, ratio of Apo A-I level to Apo A-II level was almost normal.
