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PLS is a rare autosomal recessive disease in which DPP1 insufficiency, which is caused by a mutation in the DPP1 gene ( CTSC ), results in diffuse palmoplantar hyperkeratosis, severe prepubertal periodontitis, and premature loss of both deciduous and permanent teeth ( Characterization of NSPs from patients with PLS revealed a DPP1-independent processing and maturating pathway for NSPs that is catalyzed by at least one DPP1-like protease ( In the human promyelocytic HL-60 precursor cell line, CatS/CatL inhibition nearly abolishes proDPP1 maturation, although it does not result in significant NSP inactivation

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