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Table 3 Semiquantitative evaluation of signal intensities in immunolocalization studies

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

Protein

A1

A2

A3

Z-disc (−associated) proteins

 Myotilin

↑↑↑

↓

↓∕-

 Filamin C

↑↑∕↑↑↑

↑↑↑

↑↑∕↑↑↑

 N-RAP

↑↑

↑↑↑

↑↑

 XIRP2

↑↑∕↑↑↑

↑↑↑

↑

 Xin

↑↑∕↑↑↑

↑↑↑

(↑)

 Obscurin

↑↑

↓

↑↑↑

 Myopalladin

↑↑∕↑↑↑

↑↑↑

↑∕↑↑

 Desmin

↑↑↑

(↑)

↑↑↑

 Plectin

↑

↓

↑↑↑

 Nestin

↑∕↑↑

-

↑↑↑

 Syncoilin

↑∕↑↑

↑↑↑

↑∕↑↑

Chaperone-assisted selective autophagy (CASA)/NBR1-mediated selective autophagy

 BAG3

↑↑∕↑↑↑

-

↑↑↑

 HSPB8/Hsp22

↑↑ ∕ ↑↑↑

↑↑↑

↑↑↑

 HSPA8/Hsc70

↑↑

↑

↑↑↑

 p62

↑↑↑

(↑)∕↑↑

(↑)

 NBR1

↑↑∕↑↑↑

↑

↑↑∕↑↑↑

Further chaperones

 αB-crystallin

↑∕↑↑

↑

↑↑↑

 HSPB1/Hsp27

↑∕↑↑

↑↑↑

↑↑↑

 Hsp70

↑↑

↑

↑↑↑

Unfolded protein response (UPR) / oxidative stress response/transcription regulation

 BiP

↑↑

↑↑

(↑)

 Thrombospondin 4

↑↑∕↑↑↑

↑↑↑

↑↑

 MGST3

↑↑↑

-

↑↑↑

 YBX3

↑ ∕ ↑↑

↑

↑

Actin dynamics

 MURC

(↑) ∕ ↑↑

(↑)

↑↑↑

 MYBPH

(↑) ∕ ↑↑↑

↑↑↑

(↑)

 Tropomodulin 1

↑↑ ∕ ↑↑↑

↑↑↑

(↑)

 CSRP3

↑

↑↑

↑

Microtubule organization / vesicle transport

 α-tubulin

↑↑

↑

↑↑↑

 MAP4

↑↑ ∕ ↑↑↑

(↑)

↑↑↑

 PACSIN3

(↑) ∕ ↑↑

-

↑↑↑

Sarcolemmal proteins

 Dystrophin

↑↑

-

↑↑ ∕ ↑↑↑

 Dysferlin

↑↑ ∕ ↑↑↑

↑

↑↑ ∕ ↑↑↑

 Supervillin

↑ ∕ ↑↑

-

↑↑↑

 δ-Sarcoglycan

(↑)

(↑)

(↑)

  1. A1: abnormal area 1, A2: abnormal area 2, A3: abnormal area 3; (↑)∕↑∕↑↑∕↑↑↑: slightly/moderately/distinctly/strongly increased immunoreactivity compared to control fibers; ↓: decreased immunoreactivity compared to control fibers; −: no visible immunoreactivity