Viable Mouse Models of Acid β-Glucosidase Deficiency
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چکیده
منابع مشابه
Specific saposin C deficiency: CNS impairment and acid β-glucosidase effects in the mouse
Saposins A, B, C and D are derived from a common precursor, prosaposin (psap). The few patients with saposin C deficiency develop a Gaucher disease-like central nervous system (CNS) phenotype attributed to diminished glucosylceramide (GC) cleavage activity by acid beta-glucosidase (GCase). The in vivo effects of saposin C were examined by creating mice with selective absence of saposin C (C-/-)...
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The development and recent approval of recombinant acid alpha-glucosidase for enzyme replacement therapy have been major milestones in Pompe disease research. Acid alpha-glucosidase is the enzyme responsible for degradation of glycogen polymers to glucose in the acidic milieu of the lysosomes. Cardiac and skeletal muscles are the two major tissues affected by the accumulation of glycogen within...
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A soil microorganism producing αand β-glucosidase inhibitors was identified as Bacillus lentimorbus, based on the fatty acid and morphological analyses, along with biochemical and physiological tests. The α-glucosidase inhibitor was highly produced by this strain in a culture medium containing 0.25% of sodium glutamate and 0.5% of glucose, pH 8.0 at 30C for 2 days. The α-glucosidase inhibitor f...
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ژورنال
عنوان ژورنال: The American Journal of Pathology
سال: 2003
ISSN: 0002-9440
DOI: 10.1016/s0002-9440(10)63566-3