Table 8 Statistical tests, group sizes and p-values.

1 C

CLN7 ICC (CTCF)

6

iNPC^WT = 6

iNPC^BD1 = 6

iNPC^BD2 = 6

One-way ANOVA

iNPC^WT vs iNPC^BD1 = 0.0436

2B

CLN7 (CTCF)

3

iNPC^WT = 3

iNPC^BD1 = 3

iNPC^BD2 = 3

One-way ANOVA

iNPC^WT vs iNPC^BD1 = 

iNPC^WT vs iNPC^BD2 = 

iNPC^WT + Baf vs iNPC^BD1 + Baf =

iNPC^WT + Baf vs iNPC^BD2 + Baf =

2 C

LysoTracker (CTCF)

3

iNPC^WT = 3

iNPC^BD1 = 3

iNPC^BD2 = 3

One-way ANOVA

iNPC^WT vs iNPC^BD1 = 

iNPC^WT vs iNPC^BD2 = 

3E

qRT-PCR

3

iNPC^WT = 3

iNPC^BD1 = 3

iNPC^BD2 = 3

Unpaired t-test

iNPC^BD1 vs iNPC^WT: CHCHD2, p < 0.0001, TOX2, p = 0.035, CRYAB, p < 0.0001, S100A4, p < 0.0001, FABP3, p < 0.0001, IFG2, p < 0.0001, IGF2R, p < 0.0001, IGFBP3, p < 0.0001, IGFBP5, p = 0.0002, IGFBP6, p = 0.093

iNPC^BD2 vs iNPC^WT: CHCHD2, p < 0.0001, TOX2, p + 0.0002, CRYAB, p = 0.0479, S100A4, p = 0.0086, FABP3, p < 0.0001, IFG2, p < 0.0001, IGF2R, p = 0.8266, IGFBP3, p < 0.0001, IGFBP5, p = 0.0176, IGFBP6, p < 0.0001

4B

Autophagy ICC (CTCF)

6

iNPC^WT = 6

iNPC^WT + Baf = 6

iNPC^BD1 = 6

iNPC^BD1 + Baf = 6

One-way ANOVA

Listed on graphs

5B

Mito Mem Pot

8

iNPC^WT = 6

iNPC^BD1 = 6

Two-tailed t-test

iNPC^WT vs NPC^BD1 = 0.0003, ***

5 C

MitoSOX

8

iNPC^WT = 6

iNPC^WT + Baf = 6

iNPC^BD1 = 6

iNPC^BD1 + Baf = 6

Two-tailed t-test

iNPC^WT vs iNPC^BD1 < 0.0001, ****

5D

MitoStress Test

6

iNPC^WT = 6

iNPC^WT + Baf = 6

iNPC^BD1 = 6

iNPC^BD1+ Baf = 6

Unpaired t-test

Basal Respiration

iNPC^WT +Baf vs iNPC^BD1 +Baf = 0.0007, ***

Cln7^WT +Baf vs Cln7^KO +Baf = 0.013, *

Maximal Respiration

Cont NPC +Baf vs iNPC^BD1 +Baf = 0.0066, **

Cln7^WT +Baf vs Cln7^KO +Baf = 0.0450, *

Proton Leak

iNPC^WT +Baf vs iNPC^BD1 +Baf = 0.0045, **

Cln7^WT +Baf vs Cln7^KO +Baf = 0.0043, **

ATP Production

iNPC^WT +Baf vs iNPC^BD1 +Baf = 0.0012, **

Cln7^WT +Baf vs Cln7^KO +Baf = 0.0452, *

5E

NADH/NAD+ Assay

6

iNPC^WT = 6

iNPC^WT + Baf = 6

CLN7 NPC = 6

CLN7 NPC + Baf = 6

One-tailed t-test

iNPC^BD1 vs iNPC^BD1 +Baf = 0.021, *

iNPC^WT +Baf vs iNPC^BD1+Baf = 1.467 × 10⁻⁴, ****

5 F

Apotracker

3

iNPC^WT = 3 (3)

iNPC^WT + Baf = 3 (3)

iNPC^BD2 = 3 (3)

iNPC^BD2+ Baf = 3 (3)

iNPC^BD1 = 3 (3)

iNPC^BD1+ Baf = 3 (3)

One-tailed t-test

iNPC^WT vs iNPC^WT +Baf p = n.s.

iNPC^BD2 vs iNPC^BD2+Baf p = 1.216×10^-5, ****

iNPC^BD1vs iNPC^BD1+Baf p = 1.684 × 10⁻⁵, ****