Essential Amino Acid Mnemonic
Gunners Please Note: Arginine is only an essential amino acid during times of positive nitrogen balance!
Maple Syrup Urine disease
- Autosomal recessive inborn error in the metabolism of the branched-chain amino acids ( "LIV" - Leucine, Isoleucine, Valine)
- Due to a deficiency of Branched-chain keto-acid dehydrogenase
- Leads to the accumulation of the branched-chain amino acids and their toxic ketoacid byproducts.
- Urine has the odor of maple syrup!!!
- Treatment: Dietary restriction of the branched-chain amino acids
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