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All main topics / Bio Chemisty / Clinical Bio Chem / Biochem quiz 1
Maple Syrup Urine disease
block in anabolisis of branched chain amino acids

can't changed branced alpha keto acids to CoA deriviatives because of lack in alpha ketoacid dehydrogenase deficiency

branched chain ketoacids create maple syrup (ketones smell sweet)

symptoms-difficult to feed, vomiting, lethargy, ketosis, urin smells like maple syrup, neuro damage,

treatment-diet of mixed pure amino acids
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Flashcard info:
Author: skunz11
Main topic: Bio Chemisty
Topic: Clinical Bio Chem
Published: 02.03.2010




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