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Figure 2 | Orphanet Journal of Rare Diseases

Figure 2

From: Arginine improves peroxisome functioning in cells from patients with a mild peroxisome biogenesis disorder

Figure 2

Thiolase and PEX1 immunoblots. The effect of 7, 14 and 21 days incubation of PEX1-G843D cells with arginine, glycerol and glutamine, on the amount of processed (41 kDa) peroxisomal thiolase and PEX1 protein (143 kDa) was studied by immunoblotting using cell homogenates. The precursor of thiolase, 44 kDa, is processed in peroxisomes to a mature peptide of 41 kDa. Control showed 98% processed thiolase, the severe cell line showed 1% processed thiolase. The faint upper band, seen in the ZS cell line with PEX1 antibody, is aspecific. Tubulin was used to confirm equal protein loading. Data shown are representative of two independent experiments and two additional PEX1-G843D cell lines (data not shown). ZS = severe PEX1-I700fsX41, C = Control.

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172