Christopher J. Thibodeaux

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...