Table 2.

Differentially expressed proteins identified in the mitochondrial proteomes of Mecp2^−/y and WT mice. In the experiments, we selected protein spots differing at least 2-fold between Mecp2^−/y and WT mice. The data of those spots coinciding with location (pI, molecular weight) and protein identity in the different experiments underwent two-tailed Student´s t-tests (significance level 5%) to rate significant genotype-related differences. For each protein, Uniprot identifier, molecular/biological function, and %Volume of the spots (%Vol, expressed as mean ± SD) are listed. Degrees of freedom for genotypic comparison of the listed proteins are 4–10.

N	Protein	Direction of Changes as Compared to WT	Gene	Uniprot ID	% Vol Wt Sample, Mecp2-/Y Sample, p-Value	Molecular/Biological Functions of Proteins
HIPPOCAMPUS
1	Pyruvate dehydrogenase E1 component subunit beta, mitochondrial	upregulated	Pdhb	Q9D051 ODPB_MOUSE	0.24 ± 0.23 (n = 6) 0.68 ± 0.17 (n = 6) p = 0.004	pyruvate dehydrogenase (acetyl-transferring) activity/glucose metabolic process
2	NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial	upregulated	Ndufs1	Q91VD9 NDUS1_MOUSE	0.20 ± 0.14 (n = 6) 0.53 ± 0.14 (n = 6) p = 0.002	2 iron, 2 sulfur cluster binding/electron transfer activity
3	NADH dehydrogenase [ubiquinone] iron- sulfur protein 8, mitochondrial	upregulated	Ndufs8	Q8K3J1 NDUS8_MOUSE	0.46 ± 0.41 (n = 6) 1.24 ± 0.66 (n = 6) p = 0.034	NADH dehydrogenase (ubiquinone) activity/mitochondrial electron transfer, NADH to ubiquinone
4	NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial	upregulated	Ndufv2	Q9D6J6 NDUV2_MOUSE	0.066 ± 0.075 (n = 4) 0.36 ± 0.12 (n = 4) p = 0.006	NADH dehydrogenase (ubiquinone) activity/mitochondrial electron transfer, NADH to ubiquinone
5	Cytochrome b-c1 complex subunit 1, mitochondrial	upregulated	Uqcrc1	Q9CZ13 QCR1_MOUSE	0.22 ± 0.20 (n = 6) 0.61 ± 0.23 (n = 6) p = 0.01	metal ion binding/mitochondrial electron transfer, ubiquinol to cytochrome c
6	ATP synthase subunit d	upregulated	Atp5pd	Q9DCX2 ATP5H_MOUSE	0.16 ± 0.12 (n = 6) 0.61 ± 0.25 (n = 6) p = 0.002	proton-transporting ATP synthase activity, rotational mechanism/proton motive force-driven mitochondrial ATP synthesis
7	Creatine kinase B-type	upregulated	Ckb	Q04447 KCRB_MOUSE	0.23 ± 0.17 (n = 6) 0.92 ± 0.70 (n = 6) p = 0.038	Kinase activity/phosphocreatine biosynthetic process
8	Prohibitin 1	upregulated	Phb	P67778 PHB1_MOUSE	0.036 ± 0.031 (n = 4) 0.23 ± 0.10 (n = 4) p = 0.011	protein heterodimerization activity/mitochondrial organization
9	Gamma-enolase	downregulated	Eno2	P17183 ENOG_MOUSE	0.92 ± 0.27 (n = 6) 0.36 ± 0.26 (n = 6) p = 0.004	Lyase/glycolysis
10	cAMP-dependent protein kinase catalytic subunit alpha	downregulated	Prkaca	P05132 KAPCA_MOUSE	0.17 ± 0.08 (n = 3) 0.037 ± 0.008 (n = 3) p = 0.046	Serine/threonine protein kinase activity
NEOCORTEX
11	Cytochrome b-c1 complex subunit 1, mitochondrial	upregulated	Uqcrc1	Q9CZ13 QCR1_MOUSE	0.07 ± 0.04 (n = 5) 0.20± 0.10 (n = 5) p = 0.039	metal ion binding/mitochondrial electron transfer, ubiquinol to cytochrome c
12	Guanine nucleotide-binding protein G(o) subunit alpha	upregulated	Gnao1	P18872 GNAO_MOUSE	0.20 ± 0.22 (n = 6) 0.70 ±0.47 (n = 6) p = 0.043	G protein-coupled receptor binding/G protein coupled receptor signaling pathway
13	Prohibitin 1	upregulated	Phb	P67778 PHB1_MOUSE	0.13 ± 0.09 (n = 6) 0.25 ± 0.05 (n = 6) p = 0.024	protein heterodimerization activity/mitochondria lorganization
14	Gamma-enolase	downregulated	Eno2	P17183 ENOG_MOUSE	0.48 ± 0.13 (n = 3) 0.11 ± 0.03 (n = 3) p = 0.009	Lyase/glycolysis
15	cAMP-dependent protein kinase catalytic subunit alpha	downregulated	Prkaca	P05132 KAPCA_MOUSE	0.28 ± 0.03 (n = 3) 0.11 ± 0.09 (n = 3) p = 0.031	Serine/threonine protein kinase activity
16	14-3-3 protein theta	downregulated	Ywhag	P61982 1433G_MOUSE	0.58 ± 0.15 (n = 4) 0.26 ± 0.14 (n = 4) p = 0.022	protein domain specific binding/protein targeting