Fig 1: AAT inhibits ADAM-17 activity in cell-free assay, shown as (A) relative fluorescence unit over time (min) and (B) IC50, of 15.3 µM, representing concentration at which AAT exerts half its maximal inhibitory effect on ADAM-17 activity. All conditions were repeated in triplicate and are shown as mean ± SD. ANOVA: p < 0.01 for all conditions compared to control.
Fig 2: Compound muscle action potential. Graph represents data shown in Table 1. One-way ANOVA and Tukey test: *** p < 0.001 vs. WT control; * p < 0.05 vs. CMT1A + vehicle. CMT1A + AAT vs. WT control is not significant. In this and all in vivo experiments, n = 3 animals per group.
Fig 3: Distribution of the respiratory parameters among the combination of bacterial loads and AAT enzyme levels groups. Respiratory parameters (A) FEV1, (B) FEV1/FVC6, (C) FVC6, (D) 6MWD, and (E) SPO2 were measured on two occasions: baseline (white bars), and 6 months post-initiation of therapy (black bars) among the three groups based on the combination between the bacterial loads and AAT enzyme levels. The * indicates that the difference between the two groups is statistically significant as determined by Wilcoxon signed rank test.
Fig 4: RNAseq data from plasma-derived and recombinant AATs were pooled, normalized, and analyzed by gene set enrichment analysis (GSEA) (shown here in inflamed cell condition). Upregulated (gray bars) and downregulated (black bars) gene families are shown according to normalized enrichment score. AAT was able to significantly modify some of these pathways (bold italic indicates pathways subjected to further analysis; see text).
Fig 5: Distribution of FVC6 throughout the treatment period in the genetically deficient and non-deficient groups. Forced vital capacity was measured at three occasions: baseline, 3 months, and 6 months post-initiation of therapy. The AAT-non-deficient group (white bars) significantly improved compared to the AAT-deficient group (black bars) at both 3 and 6 months. The * indicates that the difference between the two groups is statistically significant as determined by Independent samples Mann–Whitney U test.
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