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Autosomal recessive early onset parkinsonism is linked to three loci: PARK2, PARK6, and PARK7

Abstract.

Autosomal recessive, early onset parkinsonism (AREP) is genetically heterogeneous. Mutations in the parkin gene (PARK2 locus, chromosome 6q) account for up to 50% of AREP families. The parkin protein displays ubiquitin-ligase activity for different targets, which accumulate in the brain of patients with parkin defect and might cause neurodegeneration. Two new AREP loci (PARK6 and PARK7) have been recently mapped on chromosome 1p and confirmed in independent datasets, suggesting that both might be frequent. The three AREP forms display similar clinical phenotypes. Recruiting new families will help cloning the defective genes at PARK6 and PARK7 loci. This will contribute to unraveling the pathogenesis of AREP, and it is also expected to foster our understanding of molecular events underlying classic Parkinson's disease.

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  1. Department of Neurological Sciences, La Sapienza University, Viale dell'Università 30, I-00185 Rome, Italy, , , , , , IT

    V. Bonifati, N. Vanacore, G. Fabbrini, F. Stocchi & G. Meco

  2. Department of Clinical Genetics, Erasmus University, Rotterdam, The Netherlands. bonifati@kgen.fgg.eur.nl, , , , , , NL

    P. Rizzu, B. Oostra & P. Heutink

  3. Department of Epidemiology and Biostatistics, Erasmus University, Rotterdam, The Netherlands, , , , , , NL

    M.C.J. Dekker & C.M. van Duijn

  4. Neurogenetics Unit, IRCCS Neuromed, Pozzilli (IS), Italy, , , , , , IT

    F. Squitieri

  5. Division of Neurology, Misericordia Hospital, Grosseto, Italy, , , , , , IT

    R. Marconi

  6. Department of Neurosciences, Istituti Clinici di Perfezionamento, Milan, Italy, , , , , , IT

    A. Antonini

  7. Institute of Neurology, University of Perugia, Perugia, Italy, , , , , , IT

    P. Brustenghi & V. Gallai

  8. Division of Neurology, Boldrini Hospital, Thiene (VI), Italy, , , , , , IT

    A. Dalla Libera

  9. Institute of Neurology, University of Bari, Bari, Italy, , , , , , IT

    M. De Mari

  10. Institute of Neurology, University of Bologna, Bologna, Italy, , , , , , IT

    P. Montagna

  11. Department of Neurology, Erasmus Medical Centre Rotterdam, The Netherlands, , , , , , NL

    J.C. van Swieten

Authors
  1. V. Bonifati
  2. M.C.J. Dekker
  3. N. Vanacore
  4. G. Fabbrini
  5. F. Squitieri
  6. R. Marconi
  7. A. Antonini
  8. P. Brustenghi
  9. A. Dalla Libera
  10. M. De Mari
  11. F. Stocchi
  12. P. Montagna
  13. V. Gallai
  14. P. Rizzu
  15. J.C. van Swieten
  16. B. Oostra
  17. C.M. van Duijn
  18. G. Meco
  19. P. Heutink
Consortia

and the Italian Parkinson Genetics Network

Additional information

RID="*"

ID="*"The Italian Parkinson Genetics Network is composed of: G. Abbruzzese, A. Antonini, V. Bonifati, A. Berardelli, P. Brustenghi, M. Cannella, L. Capus, C. Colosimo, G. Coppola, A. Dalla Libera, N. De Leo, M. De Mari, G. De Michele, F. De Pandis, G. De Rosa, C. Di Roma, S. Di Rezze, G. Fabbrini, E. Fabrizio, A. Filla, E. Fincati, V. Gallai, G. Iliceto, P. Lamberti, N. Locuratolo, L. Lopiano, M. Manfredi, R. Marchese, R. Marconi, A. Mauro, E. Martignoni, P. Martinelli, G. Meco, C. Minardi, P. Montagna, G. Pezzoli, C. Scaglione, F. Squitieri, F. Stocchi, A. Tavella, V. Toni, G. Trianni, L. Vacca, N. Vanacore.

Correspondence to V. Bonifati

About this article Cite this article

Bonifati, V., Dekker, M., Vanacore, N. et al. Autosomal recessive early onset parkinsonism is linked to three loci: PARK2, PARK6, and PARK7. Neurol Sci 23 (Suppl 2), s59–s60 (2002). https://doi.org/10.1007/s100720200069

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