Paraproteins of familial MGUS/multiple myeloma target family-typical antigens: Hyperphosphorylation of autoantigens is a consistent finding in familial and sporadic MGUS/MM

Sandra Grass, Klaus Dieter Preuss, Stephan Thome, Dennis D. Weisenburger, Vinetta Witt, Jane Lynch, Florian Zettl, Lorenz Trümper, Natalie Fadle, Evi Regitz, Henry Lynch, Michael Pfreundschuh

Research output: Contribution to journalArticle

21 Scopus citations

Abstract

Paratarg-7 (P-7) is a frequent paraprotein target in monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma (MM), and Waldenström macroglobulinemia. Patients with P-7-specific paraproteins carry a hyperphosphorylated paratarg-7 (pP-7). Because pP-7 carrier state is dominantly inherited, we determined the paraprotein targets in 4 families with familial MGUS/MM. No antigenic target was identified for the paraproteins from 2 members of one family. Paraproteins from affected members of 2 other families targeted P-7, and paraproteins from 4 affected members of a fourth family targeted P-8, which is encoded by the ATG13 gene. P-8 was hyperphosphorylated in the affected family members (pP-8) and pP-8 carrier state is inherited in a dominant fashion. Six additional autoantigenic nonfamilial paraprotein targets were also hyperphosphorylated in the respective patients compared with normal controls. We conclude that paraproteins of affected members with familial MGUS/MM share family-typical hyperphosphorylated antigens and hyperphosphorylation of paraprotein targets might be a general mechanism underlying the pathogenesis of MGUS/MM.

Original languageEnglish (US)
Pages (from-to)635-637
Number of pages3
JournalBlood
Volume118
Issue number3
DOIs
StatePublished - Jul 21 2011

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Grass, S., Preuss, K. D., Thome, S., Weisenburger, D. D., Witt, V., Lynch, J., Zettl, F., Trümper, L., Fadle, N., Regitz, E., Lynch, H., & Pfreundschuh, M. (2011). Paraproteins of familial MGUS/multiple myeloma target family-typical antigens: Hyperphosphorylation of autoantigens is a consistent finding in familial and sporadic MGUS/MM. Blood, 118(3), 635-637. https://doi.org/10.1182/blood-2011-01-331454