Expression of leucine-rich repeat kinase 2 (LRRK2) inhibits the processing of uMtCK to induce cell death in a cell culture model system
Expression of leucine-rich repeat kinase 2 (LRRK2) inhibits the processing of uMtCK to induce cell death in a cell culture model system
PD (Parkinson's disease) is the most common neurodegenerative movement disorder. Mutations in LRRK2 (leucine-rich repeat kinase 2) gene are linked to the most common inherited and sporadic PD. Overexpression of LRRK2 and its mutants could induce mitochondrial-dependent neuronal apoptosis. However, the underlying mechanism remains elusive. We have identified several novel LRRK2 interacting proteins and showed that LRRK2 can interact with three components of the PTPC (permeability transition pore complex) including ANT (adenine nucleotide translocator), VDAC (voltage-dependent anion channel) and uMtCK [ubiquitous MtCK (mitochondrial creatine kinase)]. Those components have been reported to be involved in the permeability of mitochondrial membrane. We provide evidence that LRRK2 is likely to interact with uMtCK directly and expression of LRRK2 and its mutant form can suppress the processing of the immature form of uMtCK. LRRK2 expression keeps the uMtCK preprotein on the outer mitochondrial membrane instead of entering the mitochondria. In addition, the expression of both wild-type and mutant forms of LRRK2 promotes the interaction between ANT and VDAC, which plays a role in permeabilization transition pore opening. Finally, LRRK2-induced cell death can be suppressed by uMtCK. Our findings imply that LRRK2 can interact directly with uMtCK to block its entry into mitochondria and its subsequent processing, resulting in inhibition of mitochondrial energy channelling. Meanwhile, the decrease of uMtCK in mitochondria results in elevated interaction between ANT and VDAC and leads to neuronal apoptosis. Thus, our study provides the rational for clinical trials using creatine to treat PD and supports the notion of exploiting LRRK2 as a drug target for PD.
- Icahn School of Medicine at Mount Sinai United States
- Institute of Genetics and Developmental Biology China (People's Republic of)
- Chinese Academy of Sciences China (People's Republic of)
- Shandong Women’s University China (People's Republic of)
- State Key Laboratory of Molecular Developmental Biology China (People's Republic of)
Neurons, Creatine Kinase, Mitochondrial Form, Gene Expression, Apoptosis, Parkinson Disease, Protein Serine-Threonine Kinases, Leucine-Rich Repeat Serine-Threonine Protein Kinase-2, HEK293 Cells, Cell Line, Tumor, Humans, Voltage-Dependent Anion Channels, Protein Interaction Domains and Motifs, Mitochondrial ADP, ATP Translocases, Protein Processing, Post-Translational
Neurons, Creatine Kinase, Mitochondrial Form, Gene Expression, Apoptosis, Parkinson Disease, Protein Serine-Threonine Kinases, Leucine-Rich Repeat Serine-Threonine Protein Kinase-2, HEK293 Cells, Cell Line, Tumor, Humans, Voltage-Dependent Anion Channels, Protein Interaction Domains and Motifs, Mitochondrial ADP, ATP Translocases, Protein Processing, Post-Translational
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