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Table 4 Immunohistochemical findings

From: Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D

   Muscle Myotilin α-BC Desmin DYS-2 SMI-31 TDP-43 p62 Ubiquitin LAMP-2 VCP FHL1 Membr prot LC3 HSPB8 DNAJB6 Ubiquilin2 Cathepsin B Filamin C BAG3 α-actinin Teletonin Actin SERCA α-tubulin SMI310 Proteasome β-amyloid APP WIP1
FF1
  III-6 VL    +                     +  
  III-8 S ++ ++ + ++   ++ ++    +                    
  III-12 VL                              
   GLU    + +                          
   VL + + + +                       
  IV-7 GM ++ ++ + ++     ++                      
   GM ++ ++ ++ ++ ++ ++ ++ ++ ++   ++    ++ ++ +     ++ +
  IV-8 GM ++ ++ ++ +   ++ ++ ++   ++    ++ +    ++ +
  IV-9 VL + + +                     +
   VL + + +   ++ ++            ++ + +      
  IV-10 VL ++ ++ + +   + +                    
   VL ++ ++ + + + + ++    +      + + +  
FF2
  III-4 VL                            
  III-5 VL                           
  III-7 VL    + + +    +                      
FF3
  IV-1 VL        ++                     
   GL ++ ++ ++ +     ++                     
FF4
  III-2 VL ++ ++ ++ ++ ++ ++ ++ ++ ++   ++ + + ++ + ++        + +  
  IV-1 VL    + ++     ++                      
FF5
  IV-1 VL ++ ++ + + ++ ++ ++    + +     +       ++  
FF6
  IV-1 VL + + +/– +/– + + +   +   + +/–             
  1. –, normal, no immunoreactivity; +, present immunoreactivity; ++, prominent immunoreactivity