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Open access Oct 2026

Structural and biochemical characterization of disease-associated mutations in the human mitochondrial processing peptidase α-subunit.

The mitochondrial processing peptidase (MPP) is an essential heterodimeric protease responsible for cleavage of N-terminal mitochondrial targeting sequences (N-MTSs) from most nuclear-encoded mitochondrial proteins following import into the mitochondrial matrix. Pathogenic variants in the α-subunit of MPP (PMPCA) cause...

Danielle M. Simons, Si-Bei Qin, Kim Munro et al. · 0 citations
Open access Sep 2026

Evolutionary analysis of PINK1 reveals key roles of the N- and C-terminal extensions and TOM20 in folding its kinase domain

These findings support a model in which the NTE and CTE act as intramolecular determinants of PINK1 kinase stability and suggest that vertebrate PINK1 has evolved an increased dependence on the mitochondrial import machinery to maintain a functional state.

Tara Shomali, S. Veyron, J. Trempe · 0 citations

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