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Table 1 Proteolytic cleavage of autoantigens during apoptosis

From: Death, autoantigen modifications, and tolerance

Autoantigen Function Cleavage site Protease Fragment size Disease References
Actin Cytoskeleton LVID11, ELPD244 1 41,30,14 Autoimmune Hepatitis [95]
Alanyl tRNA synthetase Translation (VAPD632) GB 58 PM/DM, ILD [18*,51**]
CENP-B Centromere protein (VDSD457) GB 58,40 Scleroderma [18*,51**]
DNA-PK DNA repair DEVD2712 3 250,165 SLE, Scleroderma, [37*,96]
   VGPD2698 GB 250,150 Overlap Syndrome [69**]
Fibrillarin snoRNP protein VGPD184 GB 37 SLE, Scleroderma, Overlap Syndrome [51**]
α-Fodrin Actin binding protein ? Caspases 150,120 Sjögren's Syndrome [97]
    Calpain    
Histidyl tRNA synthetase Translation (LGPD48) GB 40 PM, ILD, DM, Overlap Syndrome [18*,51**]
Histone H3 DNA core protein RKQL20A FMDV 3Cpro 13 SLE [86]
hnRNP A1 RNA processing ? ? 32,29,16 SLE, RA, MCTD [34,98]
hnRNP C1 RNA processing ? 3,6,7 40 Scleroderma, Psoriasis [99]
hnRNP C2 RNA processing ? 3,6,7 40 Scleroderma, Psoriasis [99]
hsp-90 Stress response (DEED259) ? 54 SLE [18*,100]
Isoleucyl tRNA synthetase Translation (VTPD983) GB ? PM/DM, ILD [18*,51**]
Keratin Cytoskeleton VEVD238 3,6,7 26,22,19 GVHD, DLE [18*,100,101]
    Calpain    
Ki-67 Cell proliferation (VCTD1481) GB 167,148 SLE [18*,51**]
Ku-70 DNA replication, repair (ISSD79) GB ? SLE, PM/Scleroderma [18*,51**]
La Pol III transcription (DEHD371),(DEHD374) Not 1,2,3,8,9 45 SLE [54]
   LEED220 GB ?   [51**]
   VQFQ358G PV 3Cpro 50   [84]
Lamin A Nuclear skeleton VEID230 6 45 SLE-like Disease, APLA [102,103]
Lamin B Nuclear skeleton (EEID448) ? 45,28 SLE-like Disease, APLA [40,104]
   (VEVD231)     
Lamin C Nuclear skeleton VEID230 6 45 SLE-like Disease, APLA [102,103]
Mi-2 DNA methylation, VDPD1312 GB 75,72,48 DM [51**]
  chromatin remodeling      
Nucleolin Nucleolar RNA ? ? 16 SLE [34,105]
  binding protein ? GA 88   [70]
NuMA Mitosis ? 3,4,6,7,8 180,160 Sjögren's Syndrome [40,106]
   VATD1705 GB 175   [51**]
PARP DNA binding protein DEVD216 1,2,3,6 85,31 SLE [40,107,108]
   VDPD536 GB M (89,72,62)   [69**]
PM-Scl Exoribonuclease (VEQD252) GB 85,74 PM, Scleroderma [18*,51**]
PMS1 DNA repair ISAD496 GB 50,60 ? [51**]
PMS2 DNA repair VEKD493 GB 60,50,36 ? [51**]
RNA helicase A RNA processing, transcription (DTPD96) 3 M (120-130) SLE [109]
RNA polymerase I RNA synthesis (ICPD448) GB ? Scleroderma [18*,51**]
RNA polymerase II RNA synthesis (ITPD370) GB 190,110,92 Scleroderma [18*,51**]
SP1 Transcription NSPD584 3,7 68,45,22 UCTD [110]
SRP-72 Protein translation, (SELD617) 3 66 DM/PM [33]
  ER localization (VTPD573) GB 62   [51**]
Topoisomerase I DNA unwinding, DDVD146 EEED170 3 M (76-82) Scleroderma, PM [40,49]
  SR protein kinase PEDD123 3    
    6    
   IEAD15 GB 98,75,72   [51**]
Topoisomerase II DNA unwinding ? ? M (125-160) SLE, Fibrosing Alveolitis [40]
Transglutaminase Protein cross-linking ? 3 48 Coeliac Disease [59,60]
UBF/NOR-90 Nucleolar transcription factor ? ? M (24,32,35,55) Sjögren's Syndrome, [40]
   (VRPD220) GB ? Scleroderma [51**]
U1-70 kD RNA splicing DGPD341 3 40, 22 SLE, Scleroderma, MCTD [38,96]
   LGND409 GB 60   
Vimentin Cytoskeleton IDVD259 1,2,3,8,12 44,36,25,15 SLE, BD, RA, Sjögren's Syndrome [104,111,112,113]
   DSVD85     
   ? HIV-1 protease ?   [85]
  1. The 'Cleavage site' column lists sites that have been definitively identified, either by mutational analysis of the substrate P1 aspartic acid residue, or by peptide sequencing of proteolytic fragments. Sequences in parentheses signify untested but likely cleavage sites. The fourth column lists proteases that have been implicated in the cleavage reaction. Caspases are listed by number. The substrate is susceptible to direct cleavage by the indicated recombinant caspase or by purified calpain or GB in an in vitro cleavage assay. The fifth column signifies molecular weights (in kDa) of cleavage products as observed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. The acronyms used in this table (and not included in the abbreviations list at the start of the review) are listed in the appendix.