Journal
BIOCONJUGATE CHEMISTRY
Volume 12, Issue 4, Pages 603-615Publisher
AMER CHEMICAL SOC
DOI: 10.1021/bc010007l
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Funding
- NIGMS NIH HHS [R01 GM60184-01] Funding Source: Medline
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We describe the design and synthesis of substrate and internal standard conjugates for application in profiling enzyme activity of the enzymes alpha -D-2-deoxy-2-N-sulfonamido-glucosamine sulfamidase, alpha -D-2-deoxy-2-N-acetyl-glucosamine hydrolase, acetyl-coenzymeA: alpha -D-2-deoxy-2 -amino-glucosamine transferase, and alpha -D-2-deoxy-2-N-acetyl-glucosamine-6-sulfate sulfatase. Deficiency of any one of these enzymes results in a single clinical phenotype known as Sanfilippo syndrome. Such substrates have been proven effective in the confirmation of enzyme deficiency by a combination of affinity chromatography (AC) and electrospray ionization mass spectrometry (ESIMS), which forms the foundation for a new analytical technology (ACESIMS) of general interest and application to clinical and biomedical research.
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