Lysosomal Acid α-Glucosidase Consists of Four Different Peptides Processed from a Single Chain Precursor
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چکیده
منابع مشابه
Lysosomal acid alpha-glucosidase consists of four different peptides processed from a single chain precursor.
Pompe's disease is caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). GAA is synthesized as a 110-kDa precursor containing N-linked carbohydrates modified with mannose 6-phosphate groups. Following trafficking to the lysosome, presumably via the mannose 6-phosphate receptor, the 110-kDa precursor undergoes a series of complex proteolytic and N-glycan processing events,...
متن کاملLysosomal Acid -Glucosidase Consists of Four Different Peptides Processed from a Single Chain Precursor*
Pompe’s disease is caused by a deficiency of the lysosomal enzyme acid -glucosidase (GAA). GAA is synthesized as a 110-kDa precursor containing N-linked carbohydrates modified with mannose 6-phosphate groups. Following trafficking to the lysosome, presumably via the mannose 6-phosphate receptor, the 110-kDa precursor undergoes a series of complex proteolytic and Nglycan processing events, yield...
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We have used a peptide-based targeting system to improve lysosomal delivery of acid α-glucosidase (GAA), the enzyme deficient in patients with Pompe disease. Human GAA was fused to the glycosylation-independent lysosomal targeting (GILT) tag, which contains a portion of insulin-like growth factor II, to create an active, chimeric enzyme with high affinity for the cation-independent mannose 6-ph...
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The potent α-glucosidase inhibitor (compound I) was isolated from coffee brews by the activity-based fractionation and identified as a β-carboline alkaloid norharman (9H-pyrido[ 3.4-b]indole) on the basis of mass spectroscopy and nuclear magnetic resonance spectra ((1)H NMR, (13)C NMR, and COSY). The norharman showed a potent inhibition against α-glucosidase enzyme in a concentration dependent ...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2005
ISSN: 0021-9258
DOI: 10.1074/jbc.m404008200