{ "uniprot_canonical_ac": "A6H611-1", "ptm_annotation": [], "sequence": { "header": "sp|A6H611-1|MIPEP_MOUSE Isoform 1 of Mitochondrial intermediate peptidase OS=Mus musculus OX=10090 GN=Mipep PE=1 SV=1 CANONICAL=A6H611-1", "length": 711, "sequence": "MLLAAGARYARRLCGRGAAAALQGRTGRSCARDVSTSWSPVGAAFNVKPQSHLWNLLGERRGLFGVPELSTPEGFQVAQEEALKKTEWLVERACSTPPGPQTVLIFDELSDCLCRVADLADFVKIGHPDPAFREAAQEACRSIGTMVEKLNTNVELYQSLQRLLGDKKLMESLDAETRRVAELFMFDFEISGIHLDEEKRRRAVDLNVKILDLSNAFLMRTNFPNKIRKSLLPEHIQHHFARDGSHLIIDGLHAEASDDLVREAAYKIFLYPNADQLKCLEELLSSRDLLAKLVGYSTFSHRALQGTIAQTPETVMQFLEKLSEKLSERTRKDFKMMQGMKTKLNPQNSKLMPWDPPYYSGVIRAERYNIEPSLYCPFLSLGACMEGLNVLFNKLLGITLYAEQTFKGEVWCNDIRKLAVVHESEGLLGYIYCDFFQRANKPQQDCHFTIRGGRLKEDGSYQLPVVVLMLNLPHASRDFPTLLTPGMMENLFHEMGHAMHSMLGRTRYQHVTGTRCPTDFAEVPSILMEYFSNDYRVVSQFAKHYQTGQPLPKAMVSRLCESKKVCTAAEMQLQVFYAALDQIYHGQHPLKKSTTDILMETQEQFYGLPYVPDTAWQLRFSHLVGYGAKYYSYLMSRAVASMIWKECFLQDPFNRAAGERYRREMLAHGGGKEPMLMIQGMLQKCPSIDDFVDALVSDMNLDFETFFLDSK" }, "uniprot_id": "MIPEP_MOUSE", "enzyme_annotation": [ { "ec": "3.4.24.59", "activity": "" }, { "ec": "3.4.24.59", "activity": "Release of an N-terminal octapeptide as second stage of processing of some proteins imported into the mitochondrion." } ], "glycation": [], "keywords": [], "species": [ { "common_name": "Mouse", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "Mus musculus", "taxid": 10090 } ], "publication": [ { "reference": [ { "url": "https://www.ncbi.nlm.nih.gov/pubmed/27166947", "type": [ "PubMed" ], "id": "27166947" } ], "title": "The Chromatin Remodeling Complex Chd4/NuRD Controls Striated Muscle Identity and Metabolic Homeostasis.", "journal": "Cell metabolism", "evidence": [ { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_081712.2", "id": "NP_081712.2", "database": "RefSeq" } ], "authors": "Gomez-Del Arco P, Perdiguero E, Yunes-Leites PS, Acin-Perez R, Zeini M, Garcia-Gomez A, Sreenivasan K, Jimenez-Alcazar M, Segales J, Lopez-Maderuelo D, Ornes B, Jimenez-Borreguero LJ, D'Amato G, Enshell-Seijffers D, Morgan B, Georgopoulos K, Islam AB, Braun T, de la Pompa JL, Kim J, Enriquez JA, Ballestar E, Munoz-Canoves P, Redondo JM", "date": "2016" }, { "reference": [ { "url": "https://www.ncbi.nlm.nih.gov/pubmed/29151261", "type": [ "PubMed" ], "id": "29151261" } ], "title": "Mitochondrial intermediate peptidase is a novel regulator of sirtuin-3 activation by caloric restriction.", "journal": "FEBS letters", "evidence": [ { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_081712.2", "id": "NP_081712.2", "database": "RefSeq" } ], "authors": "Kobayashi M, Takeda K, Narita T, Nagai K, Okita N, Sudo Y, Miura Y, Tsumoto H, Nakagawa Y, Shimano H, Higami Y", "date": "2017" } ], "uniprot_ac": "A6H611", "site_annotation": [], "isoforms": [ { "url": "https://www.uniprot.org/uniprot/A6H611#A0A286YCX4-1", "isoform_ac": "A0A286YCX4-1", "locus": { "end_pos": 60784688, "strand": "-", "start_pos": 60798262, "chromosome": "14", "evidence": [ { "url": "https://www.ensembl.org/id/ENSMUST00000225043", "id": "ENSMUST00000225043", "database": "Ensembl Transcript" }, { "url": "https://www.ensembl.org/id/ENSMUSP00000153184", "id": "ENSMUSP00000153184", "database": "Ensembl Peptide" } ] }, "sequence": { "header": "tr|A0A286YCX4-1|MIPEP_MOUSE Computationally mapped isoform of Mitochondrial intermediate peptidase OS=Mus musculus OX=10090 GN=Mipep PE=1 SV=1 CANONICAL=A6H611-1", "length": 210, "sequence": "MLLAAGARYARRLCGRGAAAALQGRTGRSCARDVSTSWSPVGAAFNVKPQSHLWNLLGERRGLFGVPELSTPEGFQVAQEEALKKTEWLVERACSTPPGPQTVLIFDELSDCLCRVADLADFVKIGHPDPAFREAAQEACRSIGTMVEKLNTNVELYQSLQRLLGDKKLMESLDAETRRVAELFMFDFEISGIHLDEEKVISHGLDGLAV" } }, { "url": "https://www.uniprot.org/uniprot/A6H611#A0A286YDC5-1", "isoform_ac": "A0A286YDC5-1", "locus": { "end_pos": 60784688, "strand": "-", "start_pos": 60903424, "chromosome": "14", "evidence": [ { "url": "https://www.ensembl.org/id/ENSMUST00000225506", "id": "ENSMUST00000225506", "database": "Ensembl Transcript" }, { "url": "https://www.ensembl.org/id/ENSMUSP00000153374", "id": "ENSMUSP00000153374", "database": "Ensembl Peptide" } ] }, "sequence": { "header": "tr|A0A286YDC5-1|MIPEP_MOUSE Computationally mapped isoform of Mitochondrial intermediate peptidase OS=Mus musculus OX=10090 GN=Mipep PE=1 SV=1 CANONICAL=A6H611-1", "length": 568, "sequence": "MLLAAGARYARRLCGRGAAAALQGRTGRSCARDVSTSWSPVGAAFNVKPQSHLWNLLGERRGLFGVPELSTPEGFQVAQEEALKKTEWLVERACSTPPGPQTVLIFDELSDCLCRVADLADFVKIGHPDPAFREAAQEACRSIGTMVEKLNTNVELYQSLQRLLGDKKLMESLDAETRRVAELFMFDFEISGIHLDEEKRRRAVDLNVKILDLSNAFLMRTNFPNKIRKSLLPEHIQHHFARDGSHLIIDGLHAEASDDLVREAAYKIFLYPNADQLKCLEELLSSRDLLAKLVGYSTFSHRALQGTIAQTPETVMQFLEKLSEKLSERTRKDFKMMQGMKTKLNPQNSKLMPWDPPYYSGVIRAERYNIEPSLYCPFLSLGACMEGLNVLFNKLLGITLYAEQTFKGEVWCNDIRKLAVVHESEGLLGYIYCDFFQRANKPQQDCHFTIRGGRLKEDGSYQLPVVVLMLNLPHASRDFPTLLTPGMMENLFHEMGHAMHSMLGRTRYQHVTGTRCPTDFAEVPSILMEYFSNDYRVVSQFAKHYQTGQACCRSVLPSMTLWMPSCLT" } }, { "url": "https://www.uniprot.org/uniprot/A6H611#A6H611-1", "isoform_ac": "A6H611-1", "locus": { "end_pos": 60784688, "strand": "-", "start_pos": 60903462, "chromosome": "14", "evidence": [ { "url": "https://www.ensembl.org/id/ENSMUST00000063562", "id": "ENSMUST00000063562", "database": "Ensembl Transcript" }, { "url": "https://www.ensembl.org/id/ENSMUSP00000069840", "id": "ENSMUSP00000069840", "database": "Ensembl Peptide" } ] }, "sequence": { "header": "sp|A6H611-1|MIPEP_MOUSE Isoform 1 of Mitochondrial intermediate peptidase OS=Mus musculus OX=10090 GN=Mipep PE=1 SV=1 CANONICAL=A6H611-1", "length": 711, "sequence": "MLLAAGARYARRLCGRGAAAALQGRTGRSCARDVSTSWSPVGAAFNVKPQSHLWNLLGERRGLFGVPELSTPEGFQVAQEEALKKTEWLVERACSTPPGPQTVLIFDELSDCLCRVADLADFVKIGHPDPAFREAAQEACRSIGTMVEKLNTNVELYQSLQRLLGDKKLMESLDAETRRVAELFMFDFEISGIHLDEEKRRRAVDLNVKILDLSNAFLMRTNFPNKIRKSLLPEHIQHHFARDGSHLIIDGLHAEASDDLVREAAYKIFLYPNADQLKCLEELLSSRDLLAKLVGYSTFSHRALQGTIAQTPETVMQFLEKLSEKLSERTRKDFKMMQGMKTKLNPQNSKLMPWDPPYYSGVIRAERYNIEPSLYCPFLSLGACMEGLNVLFNKLLGITLYAEQTFKGEVWCNDIRKLAVVHESEGLLGYIYCDFFQRANKPQQDCHFTIRGGRLKEDGSYQLPVVVLMLNLPHASRDFPTLLTPGMMENLFHEMGHAMHSMLGRTRYQHVTGTRCPTDFAEVPSILMEYFSNDYRVVSQFAKHYQTGQPLPKAMVSRLCESKKVCTAAEMQLQVFYAALDQIYHGQHPLKKSTTDILMETQEQFYGLPYVPDTAWQLRFSHLVGYGAKYYSYLMSRAVASMIWKECFLQDPFNRAAGERYRREMLAHGGGKEPMLMIQGMLQKCPSIDDFVDALVSDMNLDFETFFLDSK" } } ], "pathway": [ { "url": "https://www.genome.jp/dbget-bin/get_linkdb?-t+pathway+mmu:70478", "resource": "KEGG Pathway", "id": "mmu:70478", "name": "" } ], "function": [ { "url": "https://www.uniprot.org/uniprot/A6H611#function", "annotation": "Cleaves proteins, imported into the mitochondrion, to their mature size.", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611#function", "id": "A6H611", "database": "UniProtKB" } ] }, { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_081712.2", "annotation": "MIPEP may contribute to the maintenance of mitochondrial quality during caloric restriction (CR) via activation of SIRT3. This novel mechanism of SIRT3 activation through MIPEP facilitates the elucidation of additional molecular pathways of CR.", "evidence": [ { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_081712.2", "id": "NP_081712.2", "database": "RefSeq" } ] } ], "orthologs": [ { "uniprot_canonical_ac": "Q99797-1", "protein_name": "Mitochondrial intermediate peptidase", "sequence": { "length": 713, "sequence": "MLCVGRLGGLGARAAALPPRRAGRGSLEAGIRARRVSTSWSPVGAAFNVKPQGSRLDLFGERRGLFGVPELSAPEGFHIAQEKALRKTELLVDRACSTPPGPQTVLIFDELSDSLCRVADLADFVKIAHPEPAFREAAEEACRSIGTMVEKLNTNVDLYQSLQKLLADKKLVDSLDPETRRVAELFMFDFEISGIHLDKEKRKRAVDLNVKILDLSSTFLMGTNFPNKIEKHLLPEHIRRNFTSAGDHIIIDGLHAESPDDLVREAAYKIFLYPNAGQLKCLEELLSSRDLLAKLVGYSTFSHRALQGTIAKNPETVMQFLEKLSDKLSERTLKDFEMIRGMKMKLNPQNSEVMPWDPPYYSGVIRAERYNIEPSLYCPFFSLGACMEGLNILLNRLLGISLYAEQPAKGEVWSEDVRKLAVVHESEGLLGYIYCDFFQRADKPHQDCHFTIRGGRLKEDGDYQLPVVVLMLNLPRSSRSSPTLLTPSMMENLFHEMGHAMHSMLGRTRYQHVTGTRCPTDFAEVPSILMEYFANDYRVVNQFARHYQTGQPLPKNMVSRLCESKKVCAAADMQLQVFYATLDQIYHGKHPLRNSTTDILKETQEKFYGLPYVPNTAWQLRFSHLVGYGARYYSYLMSRAVASMVWKECFLQDPFNRAAGERYRREMLAHGGGREPMLMVEGMLQKCPSVDDFVSALVSDLDLDFETFLMDSE" }, "evidence": [ { "url": "http://www.informatics.jax.org/homology/4337", "id": "4337", "database": "MGI" }, { "url": "https://omabrowser.org/oma/group/A6H611/", "id": "A6H611", "database": "OMA" } ], "common_name": "Human", "organism": "Homo sapiens", "gene_name": "MIPEP", "tax_id": 9606 } ], "interactions": [], "synthesized_glycans": [], "snv": [], "cluster_types": [ { "name": "isoformset.uniprotkb", "label": "Isoforms" }, { "name": "homologset.mgi", "label": "MGI Homologs" }, { "name": "homologset.oma", "label": "OMA Homologs" } ], "refseq": { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_081712.2", "ac": "NP_081712.2", "name": "mitochondrial intermediate peptidase precursor", "summary": "" }, "go_annotation": { "categories": [ { "go_terms": [ { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0004222", "pmid": "21873635", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "metalloendopeptidase activity", "id": "GO:0004222" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0004175", "pmid": "", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "endopeptidase activity", "id": "GO:0004175" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0046872", "pmid": "", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "metal ion binding", "id": "GO:0046872" } ], "total": 3, "name": "Molecular Function" }, { "go_terms": [ { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005739", "pmid": "18614015", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "mitochondrion", "id": "GO:0005739" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005739", "pmid": "14651853", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "mitochondrion", "id": "GO:0005739" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005759", "pmid": "", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "mitochondrial matrix", "id": "GO:0005759" } ], "total": 3, "name": "Cellular Component" }, { "go_terms": [ { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0006518", "pmid": "21873635", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "peptide metabolic process", "id": "GO:0006518" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0006627", "pmid": "21873635", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "protein processing involved in protein targeting to mitochondrion", "id": "GO:0006627" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0006508", "pmid": "21873635", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "name": "proteolysis", "id": "GO:0006508" } ], "total": 3, "name": "Biological Process" } ] }, "gene_names": [ { "url": "https://www.ncbi.nlm.nih.gov/protein/A6H611", "type": "synonym", "resource": "RefSeq", "name": "5730405E07Rik; MIP" }, { "url": "https://www.ncbi.nlm.nih.gov/protein/A6H611", "type": "synonym", "resource": "RefSeq", "name": "Mipep" }, { "url": "https://www.uniprot.org/uniprot/A6H611", "type": "recommended", "resource": "UniProtKB", "name": "Mipep" } ], "pro_annotation": [], "crossref": [ { "url": "https://bgee.org/?page=gene&gene_id=ENSMUSG00000021993", "id": "ENSMUSG00000021993", "database": "Bgee" }, { "url": "https://www.ncbi.nlm.nih.gov/gene/70478", "id": "70478", "database": "GeneID" }, { "url": "https://www.ebi.ac.uk/interpro/entry/IPR001567", "id": "IPR001567", "database": "InterPro" }, { "url": "https://www.ebi.ac.uk/interpro/entry/IPR024077", "id": "IPR024077", "database": "InterPro" }, { "url": "https://www.genome.jp/dbget-bin/get_linkdb?-t+pathway+mmu:70478", "id": "mmu:70478", "database": "KEGG Pathway" }, { "url": "http://www.informatics.jax.org/marker/MGI:1917728", "id": "1917728", "database": "MGI" }, { "url": "https://omabrowser.org/oma/group/VENTAWQ/", "id": "VENTAWQ", "database": "OMA" }, { "url": "http://www.orthodb.org/?query=642479at2759", "id": "642479at2759", "database": "OrthoDB" }, { "url": "http://pfam.xfam.org/family/PF01432", "id": "PF01432", "database": "Pfam" }, { "url": "http://purl.obolibrary.org/obo/PR_PR_A6H611", "id": "PR_A6H611", "database": "PRO" }, { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_081712.2", "id": "NP_081712.2", "database": "RefSeq" }, { "url": "https://www.uniprot.org/uniprot/A6H611", "id": "A6H611", "database": "UniProtKB" } ], "expression_disease": [], "glycosylation": [], "mutagenesis": [], "disease": [ { "synonyms": [ { "id": "MONDO:0014976", "url": "https://monarchinitiative.org/disease/MONDO:0014976", "resource": "MONDO", "name": "lethal left ventricular non-compaction-seizures-hypotonia-cataract-developmental delay syndrome", "description": "" }, { "id": "MONDO:0014976", "url": "https://monarchinitiative.org/disease/MONDO:0014976", "resource": "MONDO", "name": "combined oxidative phosphorylation deficiency caused by mutation in MIPEP", "description": "" }, { "id": "MONDO:0014976", "url": "https://monarchinitiative.org/disease/MONDO:0014976", "resource": "MONDO", "name": "combined oxidative phosphorylation deficiency type 31", "description": "" }, { "id": "MONDO:0014976", "url": "https://monarchinitiative.org/disease/MONDO:0014976", "resource": "MONDO", "name": "COXPD31", "description": "" }, { "id": "MONDO:0014976", "url": "https://monarchinitiative.org/disease/MONDO:0014976", "resource": "MONDO", "name": "combined oxidative phosphorylation deficiency 31; COXPD31", "description": "" }, { "id": "MONDO:0014976", "url": "https://monarchinitiative.org/disease/MONDO:0014976", "resource": "MONDO", "name": "MIPEP combined oxidative phosphorylation deficiency", "description": "" }, { "id": "DOID:0111488", "url": "http://disease-ontology.org/term/DOID:0111488", "resource": "Disease Ontology", "name": "COXPD31", "description": "" }, { "id": "DOID:0111488", "url": "http://disease-ontology.org/term/DOID:0111488", "resource": "Disease Ontology", "name": "lethal left ventricular non-compaction-seizures-hypotonia-cataract-developmental delay syndrome", "description": "" } ], "recommended_name": { "id": "DOID:0111488", "url": "http://disease-ontology.org/term/DOID:0111488", "resource": "Disease Ontology", "name": "combined oxidative phosphorylation deficiency 31", "description": "A combined oxidative phosphorylation deficiency characterized by global developmental delay, severe hypotonia, and left ventricular non-compaction that has_material_basis_in homozygous or compound heterozygous mutation in MIPEP on chromosome 13q12.12." }, "evidence": [ { "url": "https://www.alliancegenome.org/gene/0111488#disease-associations", "id": "0111488", "database": "Alliance of Genome Resources" } ] } ], "phosphorylation": [], "mass": { "monoisotopic_mass": 80852.0, "chemical_mass": 80852.0 }, "expression_tissue": [], "gene": [ { "url": "https://www.uniprot.org/uniprot/A6H611", "name": "Mipep", "locus": { "end_pos": 60905478, "strand": "+", "start_pos": 60784573, "chromosome": "14", "evidence": [ { "url": "https://www.ensembl.org/id/ENSMUSG00000021993", "id": "ENSMUSG00000021993", "database": "Ensembl Gene" } ] } } ], "protein_names": [ { "url": "https://www.uniprot.org/uniprot/A6H611#names_and_taxonomy", "type": "synonym", "resource": "UniProtKB", "name": "3.4.24.59" }, { "url": "https://www.uniprot.org/uniprot/A6H611#names_and_taxonomy", "type": "synonym", "resource": "UniProtKB", "name": "MIP" }, { "url": "https://www.uniprot.org/uniprot/A6H611#names_and_taxonomy", "type": "recommended", "resource": "UniProtKB", "name": "Mitochondrial intermediate peptidase" }, { "url": "https://www.ncbi.nlm.nih.gov/protein/A6H611", "type": "synonym", "resource": "RefSeq", "name": "mitochondrial intermediate peptidase precursor" } ] }