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Table 2 Proteins identified as over-represented in aggregate samples in myotilinopathy

From: New insights into the protein aggregation pathology in myotilinopathy by combined proteomic and immunolocalization analyses

  Mean proportion [‰]a   
Protein Aggregates Control Ratio p value
Desmin 52.09 14.36 3.6 6.75 × 10−6
Filamin C 31.82 17.86 1.8 6.69x10−6
Myotilin 24.05 4.51 5.3 2.91 × 10−8
Xin actin-binding repeat-containing protein 2 (XIRP2) 21.75 0.10 214.8 7.66 × 10−6
Nebulin-related-anchoring protein (N-RAP) 18.19 0.57 31.7 7.31 × 10−7
Plectin 14.27 6.75 2.1 0.0003
Collagen alpha-3(VI) chain 11.22 1.99 5.6 1.88 × 10−5
Obscurin 8.49 4.68 1.8 0.0020
Xin actin-binding repeat-containing protein 1 (Xin) 7.17 0.67 10.7 1.88 × 10−7
Alpha-crystallin B chain (αB-crystallin) 6.50 1.08 6.0 1.03 × 10−5
Nestin 5.14 0.11 47.3 7.62 × 10−6
Myosin-3 4.16 1.13 3.7 0.0043
Histone H4 3.87 1.23 3.1 3.08 × 10−5
Collagen alpha-1(VI) chain 3.73 0.80 4.7 0.0005
Myosin-binding protein H (MYBPH) 3.19 0.51 6.3 0.0029
Fibrillin-1 2.90 0.58 5.0 0.0230
Dysferlin 2.71 0.33 8.2 2.91 × 10−6
Prelamin-A/C 2.67 0.56 4.8 2.44 × 10−5
PDZ and LIM domain protein 3 2.56 1.26 2.0 0.0029
Collagen alpha-2(VI) chain 2.41 0.52 4.6 0.0004
Heat shock cognate 71 kDa protein (HSPA8/Hsc70) 2.40 0.99 2.4 0.0001
Collagen alpha-1(I) chain 2.05 0.53 3.8 0.0475
Sequestosome-1 (p62) 1.75 0.03 52.0 0.0001
Collagen alpha-2(I) chain 1.62 0.58 2.8 0.0080
Tubulin alpha-4A chain 1.51 0.79 1.9 0.0074
Heat shock protein beta-1 (HSPB1/Hsp27) 1.40 0.44 3.2 0.0173
Basement membrane-specific heparan sulfate proteoglycan core protein (Perlecan) 1.25 0.26 4.8 0.0077
Laminin subunit gamma-1 1.25 0.06 20.6 0.0001
NADH-cytochrome b5 reductase 1 1.13 0.69 1.6 0.0385
Laminin subunit beta-2 1.07 0.23 4.6 0.0110
Kelch-like protein 40 1.07 0.49 2.2 0.0207
Dystrophin 1.04 0.12 8.6 0.0004
Collagen alpha-2(IV) chain 0.97 0.31 3.1 0.0016
Heat shock 70 kDa protein 1A/1B 0.90 0.42 2.2 0.0030
ATP synthase F(0) complex subunit B1, mitochondrial 0.90 0.48 1.9 0.0121
Tubulin beta chain 0.79 0.29 2.8 0.0016
Decorin 0.79 0.08 9.7 0.0230
BAG family molecular chaperone regulator 3 (BAG3) 0.71 0.14 5.2 0.0007
Collagen alpha-1(III) chain 0.69 0.19 3.6 0.0348
Microsomal glutathione S-transferase 3 (MGST3) 0.68 0.27 2.5 0.0365
Synaptophysin-like protein 2 0.57 0.19 2.9 0.0087
Myosin-14 0.52 n.d. n.a. 0.0203
Muscle-related coiled-coil protein (MURC) 0.49 n.d. n.a. 0.0491
NADH-cytochrome b5 reductase 3 0.45 0.07 6.8 0.0010
Myopalladin 0.44 n.d. n.a. 0.0027
Microtubule-associated protein 4 (MAP4) 0.43 0.02 25.9 0.0110
Sarcolemmal membrane-associated protein 0.40 0.10 4.0 0.0131
Protein kinase C and casein kinase substrate in neurons protein 3 (PACSIN 3) 0.40 0.02 20.2 0.0041
Next to BRCA1 gene 1 protein (NBR1) 0.37 n.d. n.a. 0.0057
Supervillin 0.31 0.09 3.3 0.0315
14-3-3 protein gamma 0.30 0.08 3.8 0.0134
Tropomodulin-1 0.29 0.02 15.0 0.0019
Syncoilin 0.28 n.d. n.a. 0.0021
Voltage-dependent calcium channel subunit alpha-2/delta-1 0.28 0.02 16.7 0.0084
Histone H1.0 0.26 n.d. n.a. 0.0016
Signal recognition particle subunit SRP68 0.26 0.04 6.0 0.0258
Unconventional myosin-XVIIIa 0.26 n.d. n.a. 0.0043
Tubulin beta-4B chain 0.24 0.04 5.8 0.0013
Xaa-Pro aminopeptidase 1 0.23 n.d. n.a. 0.0018
Myeloid leukemia factor 2 0.22 n.d. n.a. 0.0021
Myosin-10 0.17 n.d. n.a. 0.0455
Elastin 0.16 n.d. n.a. 0.0465
Protein NipSnap homolog 2 0.16 n.d. n.a. 0.0254
60S ribosomal protein L12 0.16 0.03 6.3 0.0355
Heat shock protein beta-8 (HSPB8/Hsp22) 0.15 n.d. n.a. 0.0053
Epoxide hydrolase 1 0.15 n.d. n.a. 0.0461
Y-box-binding protein 3 (YBX3) 0.12 n.d. n.a. 0.0141
60S ribosomal protein L22 0.12 n.d. n.a. 0.0490
60S ribosomal protein L7 0.10 n.d. n.a. 0.0330
Collagen alpha-3(IV) chain 0.09 n.d. n.a. 0.0337
CD59 glycoprotein 0.09 n.d. n.a. 0.0333
SET-binding protein 0.09 n.d. n.a. 0.0333
78 kDa glucose-regulated protein (GRP78/BiP) 0.29 0.05 5.7 0.0526*
Cysteine and glycine-rich protein 3 (CSRP3) 0.21 0.08 2.8 0.0504*
Delta-sarcoglycan 0.07 n.d. n.a. 0.0781*
Thrombospondin-4 0.07 n.d. n.a. 0.0727*
  1. n.d. not detected, n.a. not applicable. Protein names marked in bold letters: proteins included in immunolocalization studies
  2. * p value between 0.05 and 0.1 but validated by immunofluorescence studies
  3. aper mill of total spectral counts