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Table 2 qPCR analysis of genes in cerebral hemispheres obtained from the end-stage of various LSDs

From: Induction of the type I interferon response in neurological forms of Gaucher disease

Gene

nGD

Krabbe

NPC1

Sandhoff

 

Fold change (−/− vs. +/−)

p value

Fold change (−/− vs. +/−)

p value

Fold change (−/− vs. +/−)

p value

Fold change (−/− vs. +/−)

p value

Inflammation

Gfap

6.8 ± 1.6

<0.005

2.6 ± 0.8

<0.05

2.1 ± 0.6

0.059

2 ± 0.5

<0.05

Adgre1 (F4/80)

6.6 ± 0.4

<0.001

3.9 ± 0.7

<0.005

3.6 ± 1.1

<0.05

2.7 ± 0.4

<0.05

Gpnmb

78.4 ± 15.6

<0.001

77.2 ± 45.4

<0.05

12.6 ± 4.9

<0.05

2.6 ± 0.4

<0.005

Tnf a

11.2 ± 2.5

<0.005

9.3 ± 5.12

<0.05

1.7 ± 0.3

<0.05

1.6 ± 0.2

<0.05

Il-1β

7.5 ± 4.6

<0.005

4.4 ± 1.0

<0.005

5.8 ± 1.4

<0.05

2.8 ± 0.1

<0.005

Ccl2

129.2 ± 46.3

<0.005

25.4 ± 16.1

<0.05

3.9 ± 1.2

<0.05

3.5 ± 1.5

<0.05

Ccl3

371.3 ± 131.7

<0.05

18.0 ± 9.3

<0.001

11.8 ± 3.5

<0.05

6.7 ± 1.3

<0.001

Ccl5

240 ± 19.3

<0.001

11.3 ± 2.2

<0.001

4.7 ± 1.2

<0.05

2 ± 0.3

<0.001

Il-10

1.8 ± 0.4

<0.05

2.0 ± 0.6

ns

0.9 ± 0.2

ns

1.1 ± 0.2

ns

Il-6

22.1 ± 7.0

<0.005

7.4 ± 3.1

<0.05

1.7 ± 0.8

ns

1.1 ± 0.3

ns

Anti-viral response

Ifnα

1.7 ± 0.8a

ns

2.0 ± 1.3

ns

0.9 ± 0.5

ns

1.5 ± 0.7

ns

Ifnβ

3.9 ± 1.7

<0.05

2.1 ± 1.7

ns

1 ± 0.5

ns

1 ± 0.4

ns

Irf7

130.9 ± 18.4

<0.001

7.3 ± 2.9

<0.05

4.6 ± 0.8

<0.001

1.6 ± 0.2

<0.05

Usp18

104.4 ± 13.3

<0.001

11.2 ± 2.4

<0.001

7.1 ± 0.5

<0.001

1.6 ± 0.2

<0.05

Oas1b

22.6 ± 5.6

<0.001

5. 7 ± 2.1

<0.05

2.6 ± 1.2

ns

1.1 ± 0.4

ns

Eif2ak2

11.7 ± 1.1

<0.001

3.5 ± 1

<0.05

1.9 ± 0.3

<0.05

1.4 ± 0.1

<0.05

RIPK pathway

Rip1

2.3 ± 0.4

<0.005

2 ± 0.36

<0.05

1.4 ± 0.2

<0.05

0.9 ± 0.04

ns

Rip3

9.1 ± 0.9

<0.001

2.3 ± 0.27

<0.05

1.5 ± 0.2

<0.05

0.7 ± 0.1

ns

Mlkl

9.4 ± 1.8

<0.001

4.74 ± 1.46

<0.05

1.8 ± 0.2

0.00898

1.2 ± 0.3

ns

  1. Results are expressed as fold change of −/− versus control mice and are means ± s.e.m. CT values were normalized to levels of HPRT. Genes that were elevated >2-fold with a p value of <0.05 are in bold. nGD mice (Gba flox/flox; nestin-Cre mice, 3 weeks of age, n = 4); Krabbe’s disease (Galc −/− mice, 5 weeks of age, n = 3); Niemann-Pick type C1 disease mice (NPC1−/−), 10 weeks of age, n = 3–4; Sandhoff disease (HexB −/− mice) 17 weeks of age, n = 2–3
  2. aIt should be noted that there is a significant difference in the extent of elevation of mRNA levels depending on whether a pathological area of the brain was used compared to half cerebral hemispheres. For instance, Ifnα was elevated 2.1-fold in layer V of the cortex, but 1.7-fold in a cerebral hemisphere. However, even when using whole cerebral hemispheres, it is apparent that the extent of the inflammatory response is much higher in nGD compared to the other LSDs
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Journal of Neuroinflammation

ISSN: 1742-2094