TPP1 is a member of the sedolisin family of serine proteases. The proenzyme is activated in the lysosome by auto-proteolysis where it cleaves tripeptides from the N-termini of substrates. Mutations of TPP1 are associated with late-infantile neuronal ceroid lipofuscinosis and Spinocerebellar Ataxia, Autosomal Recessive 7.
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