Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegeneration.

After 18 h of incubation, fluorescent‐labelled Aβ 1–40 was almost completely removed in normal and Mfn1 −/− or Mfn2 −/− controls, whereas it persisted at highly significant levels in Pitrm1 +/− MEFs and PITRM1 R183Q (Fig 7 A), as quantitatively ascertained by fluorescent cell sorting (Fig 7 B).

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