Table 3 Candidate surrogate endpoint genes responding ^a to CPX as a function of either mutant or wild-type CFTR ^b

2nd quadrant genes

CPX predominantly affects ΔF

A31

Retinoblastoma susceptibility

90

1.04

0.40

0.41

0.45

1.38

A5n

p55CDC

90

15.52

7.79

9.86

7.80

25.06

F7n

Beta-preprotachykinin

90

0.59

0.76

0.34

0.73

0.72

C6i

DNA polymerase alpha-subunit

105

0.75

2.18

1.31

1.50

3.56

CPX affects wt and ΔF oppositely

D4h

Transcriptional enhancer factor (TEF1) DNA

120

0.57

0.53

0.82

0.40

1.48

A4b

APC

120

2.28

1.89

3.17

0.86

9.30

A4c

Breast cancer susceptibility (BRCA2)

135

0.43

0.57

1.22

0.41

1.73

A4a

fgr protooncogene encoded p55-c-fgr protein

135

0.97

1.23

1.53

0.78

2.25

CPX predominantly affects wt

D4g

Transcription factor ETR101

180

2.78

6.07

12.45

3.33

12.44

A5b

DP2 dimerization partner of E2F

180

29.41

103.8

153.5

40.9

155.4

A1h

v-erbA related ear-2 protein

180

1.63

6.82

8.97

2.48

9.01

E1j

PDGF-alpha receptor

180

1.98

2.43

0.88

0.75

0.86

D6i

HOX A1 homeodomain protein (HOXA1)

180

2.50

3.43

1.45

0.86

1.24

4th quadrant genes

CPX predominantly affects ΔF

F6h

Interleukin-14 (IL-14)

270

2.12

5.67

3.92

6.00

1.98

F6g

Interleukin-13 (IL-13)

285

40.68

73.34

84.72

84.11

35.14

CPX affects wt and ΔF oppositely

A7l

C-1

330

1.59

1.65

3.77

2.34

3.18

CPX predominantly affects wt

E6g

Contactin 1 precursor (CNTN1)

345

1.56

0.71

0.71

1.52

0.62

D2f

DNA-binding protein TAXREB67

360

1.48

1.43

1.52

4.04

1.65