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Suppl. Tab. 1 list of antibodies and appropriate dilutions used in the study

Suppl. Tab. 1 list of antibodies and appropriate dilutions used in the study. Antigene Source Dilution immunoblot Dilution immunohistochemistry Company . Atf6 rabbit 1:400 Millipore . BiP rabbit 1:800 1:100 Genetex . Calnexin rabbit 1:1000 1:100 Genetex . Chop rabbit 1:500 Santa Cruz .

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Suppl. Tab. 1 list of antibodies and appropriate dilutions used in the study

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  1. Suppl. Tab. 1 list of antibodies and appropriate dilutions used in the study

  2. Antigene Source Dilution immunoblot Dilution immunohistochemistry Company

  3. Atf6 rabbit 1:400 Millipore

  4. BiP rabbit 1:800 1:100 Genetex

  5. Calnexin rabbit 1:1000 1:100 Genetex

  6. Chop rabbit 1:500 Santa Cruz

  7. Derl3 rabbit 1:500 Genetex

  8. Dnajb6 rabbit 1:1000 Genetex

  9. Dnajb11 rabbit 1:1000 Genetex

  10. eif2α mouse 1:300 Santa Cruz

  11. peif2α-Ser51 rabbit 1:1000 Abcam

  12. Emerin mouse 1:1000 Novocastra

  13. Fuca1 rabbit 1:500 Santa Cruz

  14. Gapdh mouse 1:1000 Genetex

  15. Grp94 rabbit 1:1000 Genetex

  16. Grp170 rabbit 1:1000 Genetex

  17. Hsp70 rabbit 1:1000 Genetex

  18. Ire1α rabbit 1:500 Genetex

  19. Lamin A/C mouse 1:1000 Vector Laboratories

  20. Lamin B1 rabbit 1:1000 1:100 Abcam

  21. Lamp1 rabbit 1:100 Abcam

  22. Lap2 rabbit 1:500 Millipore

  23. Lc3 rabbit 1:1000 Abcam

  24. Matrin-3 rabbit 1:1000 1:100 Bethyl

  25. p62 mouse 1:1000 1:100 Abcam

  26. Pdi rabbit 1:1000 1:100 Genetex

  27. Perk rabbit 1:500 1:100 Genetex

  28. pPerk rabbit 1:300 Cell Signaling

  29. Rab11 rabbit 1:800 Invitrogen

  30. Sec62 rabbit 1:1000 Tyedmers et al., 2000

  31. Tdp-43 mouse 1:1000 Anova

  32. Ubiquitin rabbit 1:1000 1:100 Dako

  33. Vcp rabbit 1:1000 Genetex

  34. MSS patient muscle wz mouse

  35. 16 weeks wz mouse

  36. 26 weeks wz mouse

  37. 52 weeks wz mouse

  38. 84 weeks

  39. Prominent Golgi app. ++ + ++ ++ ++

  40. Proliferated/ widened SR +++ ++ +++ +++ +++

  41. Vacuoles w/o content +++ ++ ++ +++ +++

  42. Autophagic material +++ + ++ +++ +++

  43. - Myelin-like +++ + ++ +++ +++

  44. - Granular + + + + +

  45. Glycogen accumulation + - - + +

  46. Alterations of mitochondrial cristae structure and inclusions of deposits +++ + ++ +++ +++

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