Reevaluation of phosphorylation sites in the Parkinson disease-associated leucine-rich repeat kinase 2

J Biol Chem. 2010 Sep 17;285(38):29569-76. doi: 10.1074/jbc.M110.127639. Epub 2010 Jul 1.

Abstract

Mutations in the leucine-rich repeat kinase 2 (LRRK2) gene have been identified as an important cause of late-onset, autosomal dominant familial Parkinson disease and contribute to sporadic Parkinson disease. LRRK2 is a large complex protein with multiple functional domains, including a Roc-GTPase, protein kinase, and multiple protein-protein interaction domains. Previous studies have suggested an important role for kinase activity in LRRK2-induced neuronal toxicity and inclusion body formation. Disease-associated mutations in LRRK2 also tend to increase kinase activity. Thus, enhanced kinase activity may therefore underlie LRRK2-linked disease. Similar to the closely related mixed-lineage kinases, LRRK2 can undergo autophosphorylation in vitro. Three putative autophosphorylation sites (Thr-2031, Ser-2032, and Thr-2035) have been identified within the activation segment of the LRRK2 kinase domain based on sequence homology to mixed-lineage kinases. Phosphorylation at one or more of these sites is critical for the kinase activity of LRRK2. Sensitive phospho-specific antibodies to each of these three sites have been developed and validated by ELISA, dot-blot, and Western blot analysis. Using these antibodies, we have found that all three putative sites are phosphorylated in LRRK2, and Ser-2032 and Thr-2035 are the two important sites that regulate LRRK2 kinase activity.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Antibodies, Phospho-Specific / genetics
  • Antibodies, Phospho-Specific / immunology
  • Blotting, Western
  • Cell Line
  • Cells, Cultured
  • Enzyme Activation / drug effects
  • Enzyme-Linked Immunosorbent Assay
  • Humans
  • Hydrogen Peroxide / pharmacology
  • Leucine-Rich Repeat Serine-Threonine Protein Kinase-2
  • Mice
  • Molecular Sequence Data
  • Mutation / genetics
  • Parkinson Disease / metabolism*
  • Phosphorylation
  • Protein Serine-Threonine Kinases / chemistry*
  • Protein Serine-Threonine Kinases / genetics
  • Protein Serine-Threonine Kinases / metabolism*
  • Sequence Homology, Amino Acid

Substances

  • Antibodies, Phospho-Specific
  • Hydrogen Peroxide
  • LRRK2 protein, human
  • Leucine-Rich Repeat Serine-Threonine Protein Kinase-2
  • Lrrk2 protein, mouse
  • Protein Serine-Threonine Kinases