Supplementary Table 1.Significantly differentially expressed proteins in p.Arg107Cys vs Wt expressing cells.

Gene name and protein description / UP/down / Ratio
AARS2 Probable alanyl-tRNAsynthetase / down / 0,88
ABHD10 Abhydrolase domain-containing protein 10 / down / 0,83
ACADS Short-chain specific acyl-CoA dehydrogenase / down / 0,20
BCAT2 Branched chain aminotransferase 2 / down / 0,71
CAD protein / down / 0,87
DLAT Dihydrolipoyllysine-residue acetyltransferaseof pyruvate dehydrogenase / down / 0,87
ECH1 Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase / down / 0,87
GFM1 Elongation factor G 1 / down / 0,77
HSDL2 Isoform 2 of Hydroxysteroid dehydrogenase-like protein 2 / down / 0,70
IDH2 Isocitrate dehydrogenase [NADP] / down / 0,79
KARS lysyl-tRNA synthetase / down / 0,84
MCCC1 Methylcrotonoyl-CoAcarboxylase subunit alpha / down / 0,79
MRPL13 39S ribosomal protein L13 / down / 0,75
MRPL19 39S ribosomal protein L19 / down / 0,86
MRPL40 39S ribosomal protein L40 / down / 0,86
NLN Neurolysin / down / 0,87
SUCLG2 Succinyl-CoAligase [GDP-forming] subunit beta / down / 0,85
TSFM Elongation factor Ts / down / 0,83
Unknown E: C3orf1 Transmembrane protein C3orf1 / down / 0,68
ABCB6 Isoform 1 of ATP-binding cassette sub-family B member 6 / UP / 1,19
AGPS Alkyldihydroxyacetonephosphatesynthase, peroxisomal / UP / 1,16
ARMC10 Isoform 4 of Armadillo repeat-containing protein 10 / UP / 1,24
ATAD1 ATPase family AAA domain-containing protein 1 / UP / 1,13
BAX Isoform Beta of Apoptosis regulator BAX / UP / 1,20
COX4I1 Cytochrome c oxidase subunit 4 isoform 1 / UP / 1,13
COX6C Cytochrome c oxidase subunit 6C / UP / 1,28
HARS2 Probable histidyl-tRNAsynthetase / UP / 1,17
IDH3B Isocitrate dehydrogenase 3, beta subunit / UP / 1,18
MRPL22 Isoform 1 of 39S ribosomal protein L22 / UP / 1,12
MRPL44 39S ribosomal protein L44 / UP / 1,17
MTHFD1L Methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like / UP / 1,26
NDUFAF2 Mimitin / UP / 1,54
PCCB Propionyl-CoAcarboxylase beta chain / UP / 1,19
Unknown C: C6orf125 OTTHUMP00000016217 / UP / 1,23
Unknown D: cDNA FLJ53381, highly similar to Monocarboxylate transporter 1 / UP / 1,23
VDAC2 Isoform 2 of Voltage-dependent anion-selective channel protein 2 / UP / 1,19

Supplementary Table 2.Significantly differentially expressed proteins in p.Arg107Cys vs untransduced cells.

Gene name and protein description / UP/down / Ratio
ACAD9 Acyl-CoA dehydrogenase family member 9 / down / 0,84
BCS1L Mitochondrial chaperone BCS1 / down / 0,87
CYC1 Cytochrome c1, heme protein / down / 0,84
GFM1 Elongation factor G 1 / down / 0,88
IDH2 Isocitrate dehydrogenase [NADP] / down / 0,75
MRPL13 39S ribosomal protein L13 / down / 0,71
MRPL37 Putative uncharacterized protein MRPL37 / down / 0,84
MRPL40 39S ribosomal protein L40 / down / 0,86
OXCT1 Succinyl-CoA:3-ketoacid-coenzyme A transferase 1 / down / 0,85
SURF1 Isoform 2 of Surfeit locus protein 1 / down / 0,77
ACADS Short-chain specific acyl-CoA dehydrogenase / UP / 2,11
AGPS Alkyldihydroxyacetonephosphatesynthase / UP / 1,15
ALDH4A1 Delta-1-pyrroline-5-carboxylate dehydrogenase / UP / 1,47
BAX Isoform Beta of Apoptosis regulator BAX / UP / 1,25
C6orf125 OTTHUMP00000016217 / UP / 1,21
COX4I1 Cytochrome c oxidase subunit 4 isoform 1 / UP / 1,13
FDXR Isoform Short of NADPH:adrenodoxinoxidoreductase / UP / 1,13
HARS2 Probable histidyl-tRNAsynthetase / UP / 1,27
MFN1 Isoform 3 of Mitofusin-1 / UP / 1,32
MRPL49 39S ribosomal protein L49 / UP / 1,14
MTHFD1L Methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like / UP / 1,12
MTHFD1L Methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like / UP / 1,13
NDUFV1 Isoform 1 of NADH dehydrogenase [ubiquinone] flavoprotein 1 / UP / 1,14
PRDX6 Peroxiredoxin-6 / UP / 1,19
VDAC1 Voltage-dependent anion-selective channel protein 1 / UP / 1,18

Supplementary Table 3.Significantly differentially expressed proteins in Wt expressing cells vs untransduced cells.

Gene name and protein description / UP/down / Ratio
ARMC10 25 kDa protein / down / 0,86
ATAD1 ATPase family AAA domain-containing protein 1 / down / 0,85
BCS1L Mitochondrial chaperone BCS1 / down / 0,82
MRPL22 Isoform 1 of 39S ribosomal protein L22 / down / 0,88
NDUFAF2 Mimitin / down / 0,65
SDHA 57 kDa protein / down / 0,80
ACADS Short-chain specific acyl-CoA dehydrogenase / UP / 10,33
BCAT2 Isoform A of Branched-chain-amino-acid aminotransferase / UP / 1,22
CAD protein / UP / 1,13
FDPS Farnesyl pyrophosphate synthetase / UP / 1,23
GFM1 Elongation factor G 1 / UP / 1,14
GRSF1 Isoform 1 of G-rich sequence factor 1 / UP / 1,23
HSDL2 Isoform 2 of Hydroxysteroid dehydrogenase-like protein 2 / UP / 1,37
KARS Lysyl-tRNAsynthetase / UP / 1,15
MRPL19 39S ribosomal protein L19 / UP / 1,15
MRPL9 39S ribosomal protein L9 / UP / 1,22
MRPS26 28S ribosomal protein S26 / UP / 1,22
RHOT1 ras homolog gene family, member T1 / UP / 1,15
SSBP1 Single-stranded DNA-binding protein / UP / 1,19
TSFM Elongation factor Ts / UP / 1,22
Unknown A: cDNA FLJ59762, highly similar to 39S ribosomal protein L9 / UP / 1,22
Unknown B: cDNA FLJ57664, highly similar to Mitochondrial 28S ribosomal protein S29 / UP / 1,13