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Table 2 Myositis-specific antibodies: target antigens and clinical associations in adult myositis patients

From: Myositis autoantibodies and clinical phenotypes

Autoantibody Immune target Function of autoantigen Clinical associations
Anti-ARS (Jo-1, PL-7, PL-12, EJ, OJ, KS, Ha, Zo) tRNA synthetases Aminoacylation of tRNAs PM
Anti-synthetase syndrome
Anti-Mi-2 NuRD subunit Gene transcription
Nucleosome remodeling
“Classic DM”
Mild disease
Anti-TIF1-γ Transcriptional intermediary factor 1γ Ubiquitination
Gene transcription
Severe DM
Cancer-associated DM
Anti-NXP-2 Nuclear matrix protein 2 Gene transcription Severe DM
Cancer-associated DM
Anti-MDA5 Melanoma differentiation-associated protein 5 Innate antiviral response Amyopathic DM
ILD
Poor prognosis
Anti-SAE SUMO-1 activating enzyme Protein sumoylation
Gene transcription
DM
Initially amyopathic DM
Anti-SRP Signal recognition particle Protein translocation across the ER Necrotizing myopathy
Anti-HMGCR 3-Hydroxy-3-methylglutaryl-CoA reductase Cholesterol biosynthesis Necrotizing myopathy
Prior statin use
  1. tRNAs transfer RNAs, PM polymyositis, NuRD nucleosome remodeling-histone deacetylase, DM dermatomyositis, ILD interstitial lung disease, SUMO-1 small ubiquitin-like modifier 1, ER rough endoplasmic reticulum