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Figure 3 | Orphanet Journal of Rare Diseases

Figure 3

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

Figure 3

Effect of arginine supplementation on peroxisomal D3-C22:0, pristanic acid β-oxidation and D3-C22:0 elongation. (A) The D3-C16:0/D3-C22:0 ratio (degradation) and (B) total amount of D3-C26:0 (elongation) was determined in three independent PEX1-G843D cell lines, incubated for 21 days with arginine. The D3-C16:0/D3-C22:0 ratio and total amount D3-C26:0 in control cells was 2.5 (±0.5) and 0.2 (±0.1) nmol/μg protein, respectively. In fibroblasts from the severe PEX1-I700fsX41 (ZS), the ratio and total amount was 0.002 (±0.001) and 4.2 (±0.1) nmol/μg protein, respectively. (C) The effect of 14 days arginine incubation on the pristanic acid β-oxidation in three PEX1-G843D cell lines (PEX1_1, PEX1_2 and PEX1_3). The pristanic acid β-oxidation in control cells was 544 (±182) pmol/hr.mg. Statistical analysis was performed with a two-tailed Student’s t-test, p-values, when grouped, <0.0001 (figure A), 0.007 (figure B) and 0.006 (figure C). Trend in improvement of D3-C22:0 degradation and pristanic acid β-oxidation is representative for a second independent experiment.

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