Synucleinopathy

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Synucleinopathy (also synucleinopathy) is a collective name for a heterogeneous group of neurodegenerative diseases that show the common pathological feature of an intracellular deposition of insoluble, misfolded α-synuclein in certain cell populations. Important representatives of this group of diseases are

  • of Parkinson's disease (engl. Parkinson's Disease , PD) with α-synuclein,
  • The Dementia with Lewy bodies (Engl. Dementia with Lewy Bodies , DLB)
  • the multiple system atrophy (engl. Multiple System Atrophy , MA) with their Prägnanztypen the striatonigral degeneration (engl. MSA Parkinsonian type , MSA-P) and the olivopontozerebellären atrophy (engl. MSA cerebellar type , MSA-C),
  • as well as the Hallervorden-Spatz syndrome , more general: Neurodegeneration with iron deposition in the brain (Engl. Neurodegeneration with Brain Iron Accumulation , NBIA)

While in PD and DLB the deposits are primarily found in neurons, in MSA they are primarily found in oligodendroglial cells. In NBIA, which is histologically characterized primarily by excessive iron deposits and axonal spheroids, α-synuclein deposits are found in both glial and neuronal cells and are referred to here as Papp-Lantos corpuscles. The latter disease in particular shows an overlap with the disease group of tauopathies due to an additional deposit of tau protein .

literature

  • Brás IC, Dominguez-Meijide A, Gerhardt E et al .: Synucleinopathies: Where we are and where we need to go . In: J Neurochem . January 2020, p. e14965 , doi : 10.1111 / jnc.14965 , PMID 31957016 .
  • Wong YC, Krainc D: α-synuclein toxicity in neurodegeneration: mechanism and therapeutic strategies. In: Nat Med . tape 23 , no. 2 , February 2017, p. 1-13 , doi : 10.1038 / nm.4269 , PMID 28170377 .
  • Wendy R. Galpern, Anthony E. Lang: Interface between tauopathies and synucleinopathies. A tale of two proteins . In: Annals of Neurology , Vol. 59 (2006), Issue 3, pp. 449-458, ISSN  0364-5134 , PMID 16489609 .
  • Erin H. Norris, Benoît I. Giasson, Virginia M. Lee: Alpha-synuclein. Normal function and role in neurodegenerative diseases . In: Current Topics in Development Biology , Vol. 60 (2004), pp. 17-54, ISSN  0070-2153 . PMID 15094295 .
  • Maria J. Martí, Eduardo Tolosa, Jaume Campdelacreu: Clinical overview of the synucleinopathies . In: Movement Disorders Unit / Supplement, Vol. 6 of September 18, 2003, pp. 21-27. PMID 14502652 .
  • Kurt A. Jellinger: Neuropathological spectrum of synucleinopathies . In: Movement Disorders Unit / Supplement, Vol. 6 of September 18, 2003, pp. 2-12. PMID 14502650 .