3.4.24.64: mitochondrial processing peptidase

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For detailed information about mitochondrial processing peptidase, go to the full flat file.

Reaction

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

Synonyms

Alpha-MPP, Atp23, Beta-MPP, EC 3.4.99.41, General mitochondrial processing peptidase, GPP, HA1523, HPP, Imp1, Imp2, inner membrane peptidase processing enzyme, Mas1, Mas2, Matrix peptidase, Matrix processing peptidase, Matrix processing proteinase, Mitochondrial chelator-sensitive protease, mitochondrial processing peptidase, mitochondrial processing peptidase-alpha protein, mitochondrial processing protease, Mitochondrial protein precursor-processing proteinase, MMP-beta, More, MPP, MPP-alpha, P-52, P-55, PEP, Plsp1, Plsp2, PMPCA, Processing enhancing peptidase, processing enhancing protein, processing peptidase, Proteinase, mitochondrial protein precursor-processing, thylakoidal processing peptidase, TPP, TTHA1264

ECTree

     3 Hydrolases

         3.4 Acting on peptide bonds (peptidases)

             3.4.24 Metalloendopeptidases

                3.4.24.64 mitochondrial processing peptidase

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Results	in table
2	Activating Compound
1985	AA Sequence
2	Application
1	CAS Registry Number
35	Cloned(Commentary)
4	Crystallization (Commentary)
10	Disease/ Diagnostics
15	General Information
6	General Stability
68	Inhibitors
43	KM Value [mM]
99	Localization
49	Metals/Ions
36	Molecular Weight [Da]
33	Natural Substrates/ Products (Substrates)
105	Organism
32	PDB ID
9	pH Optimum
2	pH Range
3	Posttranslational Modification
63	Protein Variants
39	Purification (Commentary)
10	Reaction
1	Reaction Type
96	Reference
20	Source Tissue
15	Specific Activity [micromol/min/mg]
4	Storage Stability
241	Substrates and Products (Substrate)
43	Subunits
82	Synonyms
9	Temperature Optimum [°C]
1	Temperature Range [°C]
1	Temperature Stability [°C]
30	Turnover Number [1/s]

Disease

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Disease on EC 3.4.24.64 - mitochondrial processing peptidase

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DISEASE

TITLE OF PUBLICATION

LINK TO PUBMED

Cerebellar Ataxia

Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA.

26657514

Cerebellar Ataxia

PMPCA mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia.

25808372

Cerebellar Ataxia

Reply: Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA.

26657516

Filariasis

Identification and reverse genetic analysis of mitochondrial processing peptidase and the core protein of the cytochrome bc1 complex of Caenorhabditis elegans, a model parasitic nematode.

16788047

Spinocerebellar Ataxias

A severe form of autosomal recessive spinocerebellar ataxia associated with novel PMPCA variants.

33272776

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