β-Leucine (beta-leucine) is a beta amino acid and positional isomer of L-leucine which is naturally produced in humans via the metabolism of L-leucine by the enzyme leucine 2,3-aminomutase.

[1][2][3] In cobalamin (vitamin B12) deficient individuals, plasma concentrations of β-leucine are elevated.

[3] A small fraction of L-leucine metabolism – less than 5% in all tissues except the testes where it accounts for about 33% – is initially catalyzed by leucine aminomutase, producing β-leucine, which is subsequently metabolized into β-ketoisocaproate (β-KIC), β-ketoisocaproyl-CoA, and then acetyl-CoA by a series of uncharacterized enzymes.

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