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... Sugar Urine Disease (a rare but potentially fatal metabolic disorder) lack an essential enzyme to break down BCAA and should not use BCAA injection.12.
A branched-chain amino acid (BCAA) is an amino acid having an aliphatic side-chain with a ... BCAAs are broken down effectively by dehydrogenase and decarboxylase enzymes ... in mice with ALS, current work does not show if a BCAA-enriched diet, given over a prolonged period, actually induces ALS-like symptoms.
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12 As a result, protein restriction is no longer recommended and should generally be avoided, given that it can worsen the nutritional status, without improving HE.
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