Biodistribution of TPP1 preparations 24 hours following IV administration.

*Only 82% of the oxidized proTPP1 could be autocatalytically converted to active enzyme. The dose represents total proenzyme based on concentration as determined by absorbance measurements, while % of injected activity recovered from all tissues analyzed is corrected for the amount of active enzyme (e.g., for OX-TPP1, pmol total injected*0.82). Data are expressed as pmol TPP1 per organ and also compared to levels present in control Tpp1(+/+) mice. Note that for total recovery, the sum of the TPP1 detected in all tissues analyzed 24 hours after administration is compared to the administered dose without correcting for half-life. Data normalized to protein are presented in Table S1.