L-Homocitrulline is metabolized to homoarginine through homoargininosuccinate via the urea cycle pathway and its metabolic abnormality could lead to Lysinuric Protein Intolerance (LPI).

[1]. Kato T, et al. Homocitrullinuria and homoargininuria in lysinuric protein intolerance. J Inherit Metab Dis. 1989;12(2):157-61.

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