Autoantibodies to Survival of Motor Neuron (SMN) Complex

Minoru Satoh, Jason Y F Chan, Angela Ceribelli, Edward K L Chan

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Autoantibodies to survival of motor neuron (SMN) complex immunoprecipitatean unusual set of the Sm D-E-F-G proteins with four additional proteins (130, 120, 38, and 33 kDa) but without other components (U1-70k, A, B'/B, C) of small nuclear ribonucleoproteins (snRNPs, U1RNP, and Sm antigens). In immunofluorescence, all anti-SMN sera showed nuclear dots (Cajal body) and cytoplasmic staining. Target antigens were identified as SMN complex (SMN, Gemin2, 3, and 4) that plays a critical role in the assembly of snRNPs. All three patients initially identified with anti-SMN were white females with polymyositis (PM), which seems interesting since deletion or mutation of SMN gene is known to cause a genetic neuromuscular disease, spinal muscular atrophy. In addition, about 25% of patients with anti-snRNPs also had coexisting anti-SMN complex autoantibodies. Coexistence of anti-SMN complex antibodies among anti-snRNPs-positive patients was associated with features of scleroderma and PM and less typical systemic lupus erythematosus.

Original languageEnglish
Title of host publicationAutoantibodies: Third Edition
PublisherElsevier B.V.
Pages139-144
Number of pages6
ISBN (Print)9780444563781
DOIs
Publication statusPublished - Dec 2013

Keywords

  • Anti-Sm antibodies
  • Anti-U1RNP antibodies
  • Cajal body
  • Gemin
  • Small nuclear ribonucleoproteins
  • SMN
  • SnRNPs
  • Spinal muscular atrophy
  • Survival of motor neuron

ASJC Scopus subject areas

  • Immunology and Microbiology(all)

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  • Cite this

    Satoh, M., Chan, J. Y. F., Ceribelli, A., & Chan, E. K. L. (2013). Autoantibodies to Survival of Motor Neuron (SMN) Complex. In Autoantibodies: Third Edition (pp. 139-144). Elsevier B.V.. https://doi.org/10.1016/B978-0-444-56378-1.00016-2