Clinically relevant intronic splicing enhancer mutation in myelin proteolipid protein leads to progressive microglia and astrocyte activation in white and gray matter regions of the brain

Table 1 Summary of quantitative neuropathological analysis of glial fibrillary acidic protein immunohistochemistry (GFAP IHC)

Brain region	C57BL/6 J		PLP-ISEdel		Two-way ANOVA
Brain region	2mo	4mo	2mo	4mo	Geno	Age	Inter
Cerebellum gm	100 ± 41	332 ± 83	188 ± 62	1,372 ± 457	*	*	*
Cerebellum wm	100 ± 17	225 ± 50	369 ± 53	740 ± 122	****	**	ns
Medulla	100 ± 30	123 ± 64	636 ± 77	1,551 ± 280	****	**	**
Pons	100 ± 53	99 ± 33	697 ± 83	2,111 ± 337	****	***	***
Midbrain	100 ± 39	47 ± 20	396 ± 62	1,331 ± 106	****	****	****
Thalamus	100 ± 26	107 ± 53	644 ± 173	2,031 ± 340	****	**	**
Striatum	100 ± 45	66 ± 23	87 ± 22	720 ± 163	**	**	**
Corpus callosum	100 ± 14	108 ± 19	206 ± 21	258 ± 37	****	ns	ns
Cortex	100 ± 23	190 ± 39	136 ± 20	800 ± 211	**	**	*
Hippocampus	100 ± 25	137 ± 20	87 ±7	153 ± 18	ns	*	ns

2-month-old WT mice used as 100%. Mean ± SEM for each brain region is indicated. (n = 5 to 6 per group). Abbreviations: *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001; geno = genotype; gm = gray matter; inter = interaction of age and genotype; mo = month-old; ns = not significant; wm = white matter.

ISSN: 1742-2094