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Comment :

Type  MGI:General Description  after in vivo administration of tracer dipeptides (i.e. the fluorophore-conjugated dipeptide d-Ala-Lys-AMCA and the radiolabeled dipeptide d-[3H]Phe-Ala), homozygotes show a severe reduction in the accumulation of hydrolysis-resistant dipeptides in the kidney, indicating impaired tubule reabsorption of peptide-bound amino acids