Question

These compounds diminish the uptake of aromatic amino acids by competing for transport into the blood-brain barrier. For 10 points each:
[10h] Name these aliphatic amino acids that cannot be metabolized in their namesake ketoaciduria, also known as maple syrup urine disease. The side chains of leucine, isoleucine, and valine classify them as this type of amino acid.
ANSWER: branched-chain amino acids [accept BCAAs]
[10m] In this disease, the body breaks down BCAAs to counteract rising ammonia levels, while allowing aromatic amino acid levels to stay elevated. NASH is a common cause of this disease, in which scarring limits the regeneration of a certain organ.
ANSWER: cirrhosis [prompt on end-stage liver disease]
[10e] Elevated BCAA levels are also present in this autoimmune condition characterized by the destruction of the pancreas’s beta cells. People suffering from this juvenile condition produce little to no insulin.
ANSWER: Type 1 diabetes [or T1D; prompt on juvenile diabetes]
<JZ, Biology>

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