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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