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59 % of its initial activity. Compared to free lipase, the Km values increased and decreased for SiO2-lipase and entrapped SiO2-lipase, respectively. Vmax value increased for both SiO2-lipase and entrapped SiO2-lipase.Acid-labile subunit (ALS) deficiency (ACLSD) constitutes the first monogenic defect involving a member of the Insulin-like Growth Factor (IGF) binding protein system. The lack of ALS completely disrupts the circulating IGF system. Autocrine/paracrine action of local produced IGF-I could explain the mild effect on growth. In the present work we
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