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Table 1 Study subject characteristics

From: Activation of the Keap1/Nrf2 stress response pathway in autophagic vacuolar myopathies

Subject ID Age Sex Drug Biopsy diagnosis LC3 (% positive fibers) SQSTM1 (% positive fibers) Keap1 (% positive fibers)
Normal control group
1 52 F None Normal 0.0 0.0 0.0
2 67 F None Normal 0.0 0.0 0.0
3 83 F None Normal 0.0 0.0 0.0
4 56 F None Normal 0.0 0.0 0.3
5 53 M None Normal 0.0 0.0 0.0
6 57 M None Normal 0.0 1.0 0.2
7 60 M None Normal 0.0 0.0 0.0
8 64 F None Normal 0.0 0.0 ND
9 48 M None Normal 0.0 0.0 0.0
10 32 M None Normal 0.0 0.0 0.0
Drug-treated control group
11 32 F HCQ Necrotizing myopathy 13.0 10.5 4.5
12 58 F HCQ Inflammatory myopathy 2.5 4.3 2.0
13 33 M HCQ Normal 0.3 0.2 0.5
14 65 F HCQ Neurogenic changes 4.0 2.0 1.0
15 44 F Colchicine Neurogenic changes 1.8 1.5 1.0
16 47 F HCQ Normal 0.8 0.3 1.0
17a 60 F HCQ Polymyositis; neurogenic changes 0.5 2.0 2.0
18a 59 F HCQ Normal 4.3 2.3 3.8
19a 31 F HCQ Inflammatory myopathy 0.8 0.5 2.0
Toxic autophagic vacuolar myopathy group
20 73 M Colchicine Toxic AVM 64.0 86.0 72.0
21 58 M Colchicine Toxic AVM 22.5 14.8 8.0
22 81 F Colchicine Toxic AVM 78.0 83.5 87.0
23 84 M Colchicine Toxic AVM 58.5 65.0 60.0
24 80 M Colchicine Toxic AVM 13.0 21.0 34.0
25 72 F HCQ Toxic AVM 56.5 53.0 45.5
26 33 F HCQ Toxic AVM 18.0 12.5 13.5
27 79 M Colchicine Toxic AVM 79.0 79.5 49.0
28 79 M Colchicine Toxic AVM 95.0 93.0 78.0
29 29 M HCQ Toxic AVM 12.5 25.5 31.5
30a 62 F Colchicine Toxic AVM 21.8 27.3 37.3
31a 66 M Colchicine Toxic AVM 17.5 18.3 10.5
32a 24 F HCQ Toxic AVM 10.5 12.5 14.0
Polymyositis group
33 59 M None Polymyositis 5.7 5.5 5.0
34 85 M None Polymyositis 6.0 10.0 6.0
35 51 F None Polymyositis 4.0 0.3 0.5
36 65 M None Polymyositis 2.5 3.7 1.5
37 47 M None Polymyositis 1.7 11.0 9.0
38 42 M None Polymyositis 11.3 10.2 3.0
39 66 F None Polymyositis 3.8 4.0 0.5
40 59 M None Polymyositis 6.7 8.3 5.5
41 53 M None Polymyositis 2.7 19.2 12.5
42 43 M None Polymyositis 2.7 4.0 1.5
43 50 M None Polymyositis 2.0 2.8 0.5
Inclusion body myositis group
44 64 F None IBM 31.0 18.5 24.0
45 57 F None IBM 24.0 20.0 34.0
46 62 M None IBM 29.5 59.5 48.5
47 77 M None IBM 15.5 16.5 15.5
48 74 F None IBM 16.0 9.0 7.0
49 73 F None IBM 34.0 32.0 24.0
50 69 F None IBM 48.5 43.5 20.5
51 75 M None IBM 11.0 22.5 20.0
52 59 M None IBM 33.5 36.0 40.0
53 66 M None IBM 9.0 15.0 6.5
54 76 M None IBM 32.5 12.0 27.5
55b 80 M None IBM 9.5 13.5 11.8
  1. ND Not determined (FFPE tissue was exhausted, but the subject was retained in the cohort because frozen tissue for qRT-PCR analysis was available)
  2. aSubject added to the cohort after the publication of reference [11]
  3. bSubject added to the cohort after the publication of reference [15]
  4. Note: This table includes only the information regarding treatment with autophagy inhibiting drugs (HCQ and colchicine), not the full list of patient medications