THE HURLER SYNDROME TABLE II Intracellular Mucopolysaccharides in Cztltured Lymphoid and Skin Fibroblast Lines Derived from a Patient with the Hurler Syndrome

The Hurler syndrome is a genetic disorder in mucopolysaccharide metabolism which results in storage of mucopolysaccharides in various tissues of the body (1). The lymphocytes in the peripheral blood of patients with the Hurler syndrome show metachromatic inclusions which are considered to be diagnostic (2). I t has not been established whether these inclusions reflect the uptake of mucopolysaccharides from the plasma or synthesis by the bTnphocytes. With the development of techniques for the establishment and maintenance of permanent l~nnphoid cell lines (3), it has been possible to study the mucopolysaccharide metabolism of such suspension cell lines derived from cells of the peripheral blood of both pat ients with the Hurler syndrome and normal individuals in an a t tempt to answer such questions.