Table 1 Correlation between the proliferation, sXBP1 and ATF6 expression and peIF2α levels in CDG and CDDG cell lines treated with candidate and non-active control compounds.

From: Precision genetic cellular models identify therapies protective against ER stress

Compounds

NGLY1/−

PMM2F119L/−

DPAGT1+/−

Proliferation

sXBP1

peIF2α

ATF6

Proliferation

sXBP1

peIF2αa

ATF6a

Proliferation

sXBP1

peIF2α

ATF6

Candidates Group I

1

**

ns

  

ns

ns

  

ns

ns

  

2

****

ns

  

ns

ns

  

ns

ns

  

3

ns

****

  

***

ns

  

****

*

  

4

ns

****

  

ns

ns

  

ns

**

  

5

ns

*

  

*

ns

  

ns

ns

  

6

****

****

****

***

****

**

ns

ns

***

**

****

****

9

***

****

****

*

***

**

ns

ns

****

***

****

****

Candidates Group II

14

ns

****

  

ns

ns

  

ns

***

  

15

****

****

****

**

****

**

ns

ns

****

****

****

****

16

***

ns

  

ns

ns

  

ns

ns

  

18

****

ns

  

ns

ns

  

ns

ns

  

25

ns

****

  

****

**

  

****

****

  

26

ns

****

****

**

****

***

ns

ns

****

****

****

***

Non-active controls

28

ns

ns

ns

ns

ns

ns

ns

ns

ns

ns

****

ns

29

ns

ns

  

ns

ns

  

ns

ns

  

30

ns

ns

  

ns

ns

  

ns

ns

  

31

ns

ns

  

ns

ns

  

ns

ns

  

32

ns

ns

  

ns

ns

  

ns

ns

  

33

ns

ns

  

ns

ns

  

ns

ns

  

34

ns

ns

  

ns

ns

  

ns

ns

  

35

ns

ns

  

ns

ns

  

ns

ns

  

36

ns

ns

  

ns

ns

  

ns

ns

  

37

ns

ns

  

ns

ns

  

ns

ns

  

38

ns

ns

  

ns

ns

  

ns

ns

  
  1. aNo phenotype for these ER stress markers.
  2. *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001.