Intranuclear aggregation of nonexpanded ataxin-3 in Marinesco bodies of the nonhuman primate substantia nigra

Mattias Kettner, Dirk Willwohl, Gene B. Hubbard, U. Rüb, Edward J. Dick, Ann B. Cox, Yvon Trottier, Georg Auburger, Heiko Braak, Christian Schultz

Research output: Contribution to journalArticlepeer-review

24 Scopus citations


Marinesco bodies (MB) are intranuclear inclusion bodies predominantly found in melanin-pigmented neurons of the substantia nigra. MB are demonstrable not only in humans but also in nonhuman primates. In the present study MB of aged rhesus monkeys (Macaca mulatta; n = 15; mean age 16 years) and aged baboons (Papio anubis; n = 13; mean age 25 years) were examined immunohistochemically. MB were found to be immunoreactive for ubiquitin, a protein involved in initiation of proteasome-mediated proteolysis. We also demonstrate that MB in monkeys are intensely immunoreactive for the protein ataxin-3 as detected by using two monoclonal anti-ataxin-3 antibodies (1H9 and 2B6). The abnormally expanded form of this polyglutamine protein is known to be causally involved in spinocerebellar ataxia type 3 or Machado-Joseph disease. The monoclonal antibody 1C2 was employed to examine whether ataxin-3 in MB in monkeys contains such an abnormally expanded polyglutamine stretch. MB were consistently 1C2-immunonegative, indicating that they are composed of normal wild-type ataxin-3. In conclusion MB in nonhuman primates permit experimental examination of mechanisms involved in transnuclear localization, intranuclear aggregation, and ubiquitination of nonexpanded polyglutamine proteins.

Original languageEnglish (US)
Pages (from-to)117-121
Number of pages5
JournalExperimental Neurology
Issue number1
StatePublished - 2002
Externally publishedYes


  • Ataxin-3
  • Intranuclear inclusion
  • Marinesco bodies
  • Nonhuman primates
  • Polyglutamine disease
  • Spinocerebellar ataxia
  • Ubiquitin

ASJC Scopus subject areas

  • Neurology
  • Developmental Neuroscience


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