Table 1 Passive membrane properties

From: Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

 

Control

TARDBP

C9ORF72

Cm (pF)

 Weeks 3–4

10.4±0.4 (196)

12.0±0.5 (103)

8.7±0.3 (102)##

 Weeks 5–6

11.6±0.3 (322)

11.2±0.4 (171)

10.1±0.5 (69)

 Weeks 7–8

10.7±0.3 (216)

16.1±1.1 (96)***

11.9±0.9 (52)##

 Weeks 9–10

14.0±0.6 (111)

19.7±1.2 (82)***

14.5±0.7 (42)##

RN(MΩ)

 Weeks 3–4

727±22

660±33

723±33

 Weeks 5–6

774±21

788±27

768±42

 Weeks 7–8

774±23

762±38

772±45

 Weeks 9–10

709±30

688±39

643±58

RMP(mV)

 Weeks 3–4

−45.6±0.8

−39.9±1.0**

−44.8±1.2

 Weeks 5–6

−42.8±0.7

−40.4±1.0

−44.2±1.5

 Weeks 7–8

−45.6±0.8

−43.2±1.4

−37.9±1.5***

 Weeks 9–10

−49.8±1.1

−40.1±1.9***

−45.2±1.9

  1. Values are mean ±s.e.m.; number of cells noted in parentheses.
  2. Significantly different to controls (**P<0.001; ***P<0.0001).
  3. Significant difference between patient lines (##P<0.001).
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