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Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Proc+Natl+Acad+Sci+U+S+A 2018 ; 115 (2): 409-14 Nephropedia Template TP
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Familial Parkinson?s point mutation abolishes multiple system atrophy prion replication #MMPMID29279394
Proc Natl Acad Sci U S A 2018[Jan]; 115 (2): 409-14 PMID29279394show ga
In Parkinson?s disease (PD) and multiple system atrophy (MSA), the accumulation and spread of ?-synuclein prions leads to the progressive degeneration seen in patients. These diseases are thought to arise from unique conformations of ?-synuclein prions?or strains. To investigate this hypothesis, we infected cell lines expressing PD-causing point mutations in ?-synuclein with MSA patient samples. Unexpectedly, the E46K mutation inhibited MSA prion replication. This observation indicates that the ?-synuclein prion conformation found in PD is different from the strain found in MSA, providing biological evidence that these diseases arise from distinct prion strains. The ability of ?-synuclein recombinant fibrils to infect all cell lines tested argues that synthetic ?-synuclein prions are not predictive of the disease-causing strains in human patients.