Mitochondrial processing peptidases.
Three peptidases are responsible for the proteolytic processing of both nuclearly and mitochondrially encoded precursor polypeptides targeted to the various subcompartments of the mitochondria. Mitochondrial processing peptidase (MPP) cleaves the vast majority of mitochondrial proteins, while inner membrane peptidase (IMP) and mitochondrial intermediate peptidase (MIP) process specific subsets of precursor polypeptides. All three enzymes are structurally and functionally conserved across species, and their human homologues begin to be recognized as potential players in mitochondrial disease.
Departments of Pediatric & Adolescent Medicine and Biochemistry & Molecular Biology, Mayo Clinic and Foundation, 200 First Street SW, Stabile 7-48, Rochester, MN 55905, USA.
Oleksandr
O
Gakh
Patrizia
P
Cavadini
Grazia
G
Isaya
D000595:Amino Acid Sequence; D000818:Animals; D001665:Binding Sites; D004926:Escherichia coli; D006801:Humans; D007425:Intracellular Membranes / Q000201:enzymology*; D008666:Metalloendopeptidases / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D008928:Mitochondria / Q000201:enzymology*; D024101:Mitochondrial Proteins / Q000737:chemistry / Q000378:metabolism; D008969:Molecular Sequence Data; D010802:Phylogeny; D011498:Protein Precursors / Q000378:metabolism*; D012441:Saccharomyces cerevisiae; D016415:Sequence Alignment; D013329:Structure-Activity Relationship; D013379:Substrate Specificity; D000096122:Mitochondrial Processing Peptidase
D008666:Metalloendopeptidases; D024101:Mitochondrial Proteins; D011498:Protein Precursors; D000096122:Mitochondrial Processing Peptidase; C068038:mitochondrial intermediate peptidase