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Table 2 The distribution of OPMD-deregulated genes in UPS functional units and protein degradation categories.

From: Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients

  Drosophila Mouse Human Overlap mouse vs. human
  # Total Genes % D.E. Genes P Value (FDR) # Total Genes % D.E. Genes P -Value (FDR) # Total Genes % D.E. Genes P -Value (FDR) # D.E. Genes % D.E. Genes
Ubiquitin 2 50.00 4.18E-05 3 11.11 1.28E-01 3 33.33 1.19E-01 0 00.00
E1 Ubiquitin Activation 4 16.67 1.24E-01 7 04.76 7.29E-02 7 14.29 7.94E-02 0 00.00
E2 Ubiquitin Conjugation 22 13.64 9.19E-06 33 44.42 1.51E-08 34 23.53 7.31E-02 3 37.50
E3 Ubiquitin Ligase 249 13.99 1.92E-04 526 29.58 1.64E-08 586 24.74 4.35E-03 69 47.59
Deubiquitination (DUB) 35 20.00 1.63E-05 72 45.83 1.48E-08 75 24.00 3.15E-02 13 72.22
¥ Proteasome 47 29.79 2.15E-04 37 36.94 1.37E-07 37 51.35 9.27E-03 11 57.90
¥ Autophagy 16 25.00 1.07E-03 39 30.77 8.13E-08 32 18.75 1.37E-02 1 16.67
¥ Lysosome 60 5.00 1.64E-02 75 25.33 6.06E-03 73 24.68 1.54E-02 6 33.33
  1. The number of annotated genes per unit, the percentage of OPMD-deregulated (D.E.) genes and P- values are indicated per organism. For Drosophila and mouse statistics is generated in combined datasets from three time points. The overlap in OPMD-deregulated genes between humans and mice and the percentage of deregulated genes in human D.E. genes are indicated. Protein degradation machineries are depicted by ¥.