3.4.24.59: mitochondrial intermediate peptidase

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

Reaction

release of an N-terminal octapeptide as second stage of processing of some proteins imported into the mitochondrion =

Synonyms

At1g09300, hMIP, Icp55, MIP, MIP1, MIPEP, mitochondrial intermediate peptidase, OCT1, octapeptidyl aminopeptidase 1, Proteinase, mitochondrial intermediate precursor-processing

ECTree

     3 Hydrolases

         3.4 Acting on peptide bonds (peptidases)

             3.4.24 Metalloendopeptidases

                3.4.24.59 mitochondrial intermediate peptidase

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Results	in table
1	Activating Compound
3275	AA Sequence
1	Application
1	CAS Registry Number
9	Cloned(Commentary)
11	Disease/ Diagnostics
5	General Information
33	Inhibitors
15	kcat/KM [mM/s]
3	Ki Value [mM]
15	KM Value [mM]
14	Localization
11	Metals/Ions
10	Molecular Weight [Da]
8	Natural Substrates/ Products (Substrates)
21	Organism
3	pH Optimum
1	pH Range
2	Protein Variants
7	Purification (Commentary)
1	Reaction
1	Reaction Type
21	Reference
11	Source Tissue
2	Specific Activity [micromol/min/mg]
2	Storage Stability
103	Substrates and Products (Substrate)
8	Subunits
26	Synonyms
2	Temperature Optimum [°C]
14	Turnover Number [1/s]

Disease

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Disease on EC 3.4.24.59 - mitochondrial intermediate peptidase

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DISEASE

TITLE OF PUBLICATION

LINK TO PUBMED

Muscle Hypotonia

MIPEP recessive variants cause a syndrome of left ventricular non-compaction, hypotonia, and infantile death.

27799064

Myopia

Association of the ZC3H11B, ZFHX1B and SNTB1 genes with myopia of different severities.

31300455

Myopia

Genetic variants at 13q12.12 are associated with high myopia in the Han Chinese population.

21640322

Myopia

Genotype-phenotype correlation and interaction of 4q25, 15q14 and MIPEP variants with myopia in southern Chinese population.

31604699

Neurodegenerative Diseases

Functional and genomic analysis of the human mitochondrial intermediate peptidase, a putative protein partner of frataxin.

10783257

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