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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