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. 2011 Jul 5;19(10):1842–1848. doi: 10.1038/mt.2011.130

Figure 1.

Figure 1

Uptake of intravenously administrated recombinant human TPP1 (rhTPP1) and effect on subunit c of mitochondrial ATP synthase (SCMAS) storage in liver and brain. Fifteen week-old Tpp1(−/−) mice were killed 24 hours after administration of 100 µl artificial cerebrospinal fluid (CSF) (vehicle) or 12 mg/ml rhTPP1 (enzyme) and compared to age-matched wild type controls. Equal amounts of each sample (3 µg protein) were analyzed by immunoblotting. Relative intensities (mean ± SD) of SCMAS staining in each tissue for vehicle-treated (n = 3), enzyme-treated (n = 3), and wild-type controls (n = 2), respectively, were as follows: Liver- 1.00 ± 0.26, 0.53 ± 0.06, 0.35 ± 0.03; Brain- 1.00 ± 0.13, 1.03 ± 0.04, 0.37 ± 0.05. Note that this signal includes both normal mitochondrial and stored SCMAS.