3.4.24.64: mitochondrial processing peptidase

This is an abbreviated version!
For detailed information about mitochondrial processing peptidase, go to the full flat file.

Word Map on EC 3.4.24.64

Reaction

Release of N-terminal targetting peptides from precursor proteins imported into the mitochondrion, typically with Arg in position P2 =

Synonyms

More, Processing enhancing peptidase, Proteinase, mitochondrial protein precursor-processing, Matrix peptidase, Matrix processing peptidase, Matrix processing proteinase, Mitochondrial protein precursor-processing proteinase, MPP, Mitochondrial chelator-sensitive protease, General mitochondrial processing peptidase, TPP, processing peptidase, Alpha-MPP, Beta-MPP, HA1523, P-52, P-55, PEP, processing enhancing protein, Mas1, Mas2, Plsp1, Atp23, inner membrane peptidase processing enzyme, Imp1, Imp2, mitochondrial processing peptidase, HPP, GPP, TTHA1264, thylakoidal processing peptidase, Plsp2, MPP-alpha, MMP-beta, mitochondrial processing peptidase-alpha protein, PMPCA, mitochondrial processing protease

ECTree

     3 Hydrolases
         3.4 Acting on peptide bonds (peptidases)
             3.4.24 Metalloendopeptidases
                3.4.24.64 mitochondrial processing peptidase

Natural Substrates Products

Natural Substrates Products on EC 3.4.24.64 - mitochondrial processing peptidase

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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
COX IV precursor + H2O
processed COX IV + mitochondrial targeting sequence of COX IV
show the reaction diagram
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?
epidermal growth factor receptor preprotein + H2O
mature epidermal growth factor receptor + prepeptide of epidermal growth factor receptor
show the reaction diagram
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?
mitochondrial carrier protein + H2O
?
show the reaction diagram
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the N-terminal extension of plant mitochondrial carrier proteins is removed by two-step processing. The first cleavage is by the mitochondrial processing peptidase
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?
mitochondrial glycerol-3-phosphate dehydrogenase + H2O
processed mitochondrial glycerol-3-phosphate dehydrogenase + prepeptide
show the reaction diagram
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?
mitochondrial matrix protein precursor + H2O
mitochondrial matrix protein + precursor
show the reaction diagram
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?
Nfs1 + H2O
processed Nfs1
show the reaction diagram
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MPP cleaves the precursor between Phe33 and Tyr34, Nfs1 processing, overview
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?
nuclear-encoded polyprotein precursor + H2O
?
show the reaction diagram
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the nuclear-encoded protein RPS14 (ribosomal protein S14) of rice mitochondria is synthesized in the cytosol as a polyprotein consisting of a large N-terminal domain comprising preSDHB (succinate dehydrogenase B precursor) and the C-terminal RPS14. After the preSDHB–RPS14 polyprotein is transported into the mitochondrial matrix, the protein is processed into three peptides: the N-terminal prepeptide, the SDHB domain and the C-terminal mature RPS14. MPP (mitochondrial processing peptidase) plays an essential role in processing of the polyprotein. Purified yeast MPP cleaves both the N-terminal presequence and the connector region between SDHB and RPS14. The connector region is processed more rapidly than the presequence. The cleavage site between SDHB and RPS14 is located in an MPPprocessing motif. MPP interacts with multiple sites in the region, possibly in a similar manner to the interaction with the N-terminal presequence. In addition, MPP preferentially recognizes the unfolded structure of preSDHB–RPS14. In mitochondria, MPP may recognize the stretched poly-protein during passage of the precursor through the translocational apparatus in the inner membrane, and cleaves the connecting region between the SDHB and RPS14 domains even before processing of the presequence
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?
pea glutathione reductase + H2O
?
show the reaction diagram
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signal peptide is cleaved off by the mitochondrial processing peptidase. Removal of 30 N-terminal amino acid residues of the signal peptide (GRD1–30) greatly stimulates processing activity. Constructs with a deletion of an additional ten amino acid residues (GRD1–40) and deletion of 22 amino acid residues in the middle of the GR signal sequence (GRD30–52) are not celeaved by MPP. Mutations within two amino acid residues on either side of the processing site have inhibitory effect on processing by MPP with a nearly complete inhibition for mutations at position K1. Mutation of positively charged residues in the C-terminal half of the GR targeting peptide inhibit processing by MPP
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?
phosphatase and tensin homologue-induced kinase 1 + H2O
?
show the reaction diagram
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?
pre-F1FO-ATP synthase + H2O
mature F1FO-ATP synthase + prepeptide
show the reaction diagram
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on one hand, Atp23 serves as a processing peptidase and mediates the maturation of the mitochondrially-encoded FO-subunit Atp6 after its insertion into the inner membrane, on the other hand, independent of its proteolytic activity, Atp23 promotes the association of mature Atp6 with Atp9 oligomers with chaperone activity, overview, the assembly step is thus under the control of two substrate-specific chaperones, Atp10 and Atp23, which act on opposite sides of the inner membrane, modelling of assembly, overview
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?
presequence-containing protein + H2O
presequence + protein
show the reaction diagram
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?
processing enhancing protein precursor + H2O
processing enhancing protein + ?
show the reaction diagram
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?
additional information
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