Hahn, Steven

Several cases of a new form of metachromatic leukodystrophy have been reported (Hahn et al., 1981, 1982 Inui et al., 1983). These cases have clinical symptoms resembling juvenile metachromatic leukodystrophy, but have only about half of the normal arylsulfohydrolase A activity in leukocytes and fibroblasts. The kinetic properties of the arylsulfohydrolase A from fibroblasts are normal. However, the cerebroside 3-sulfate loading test (Porter et al., 1971a) with growing fibroblasts shows an abnormal response, indicating a disturbance in arylsulfatase A activity. Supplementation with the activator (Stevens et al., 1981) of arylsulfatase A results in a normal cerebroside 3-sulfate loading test. This indicates that the new form of metachromatic leukodystrophy is not caused by the deficiency of arylsulfohydrolase A, but rather is caused by a deficiency of the activator protein (Shapiro et al., 1979). Fujibayashi and Wenger (1986) have studied the biosynthesis of sulfa-tide/GMj activator protein in control and mutant cultured skin fibroblasts. Their results indicate that patients with variant form of metachromatic leu-... 

Hahn, M.S., Teply, B.A., Stevens, M.M., Zeitels, S.M., Langer, R., 2006. Collagen composite hydrogels for vocal fold lamina propria restoration. Biomaterials 27 (7), 1104—1109. 

Hahn, K. M. Toutchkine, A. Muthyala, R. Kraynov, V. Bark, Steven J. Burton, D. R. Chamberlain, C. Labeled peptides, proteins and antibodies and processes and intermediates useful for their preparation. PCT Int. Appl. WO 2002008245, 2002 Chem. Abstr. 2002, 136, 163716. 

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