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Aliases for MIPEP Gene

Aliases for MIPEP Gene

  • Mitochondrial Intermediate Peptidase 2 3
  • EC 4 64
  • MIP 3 4
  • HMIP 3

External Ids for MIPEP Gene

Summaries for MIPEP Gene

Entrez Gene Summary for MIPEP Gene

  • The product of this gene performs the final step in processing a specific class of nuclear-encoded proteins targeted to the mitochondrial matrix or inner membrane. This protein is primarily involved in the maturation of oxidative phosphorylation (OXPHOS)-related proteins. This gene may contribute to the functional effects of frataxin deficiency and the clinical manifestations of Friedreich ataxia. [provided by RefSeq, Jul 2008]

GeneCards Summary for MIPEP Gene

MIPEP (Mitochondrial Intermediate Peptidase) is a Protein Coding gene. Among its related pathways are Transport of glucose and other sugars, bile salts and organic acids, metal ions and amine compounds and Regulation of Water Balance by Renal Aquaporins. GO annotations related to this gene include metalloendopeptidase activity.

UniProtKB/Swiss-Prot for MIPEP Gene

  • Cleaves proteins, imported into the mitochondrion, to their mature size

No data available for Tocris Summary , Gene Wiki entry , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for MIPEP Gene

Genomics for MIPEP Gene

Genomic Location for MIPEP Gene

23,730,189 bp from pter
23,889,448 bp from pter
159,260 bases
Minus strand

Genomic View for MIPEP Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for MIPEP Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for MIPEP Gene

Regulatory Elements for MIPEP Gene

Proteins for MIPEP Gene

  • Protein details for MIPEP Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Mitochondrial intermediate peptidase
    Protein Accession:
    Secondary Accessions:
    • Q5JV15
    • Q5T9Q9
    • Q96G65

    Protein attributes for MIPEP Gene

    713 amino acids
    Molecular mass:
    80641 Da
    Name=Zn(2+); Xref=ChEBI:CHEBI:29105; Note=Binds 1 zinc ion.;
    Quaternary structure:
    • Monomer.

neXtProt entry for MIPEP Gene

Proteomics data for MIPEP Gene at MOPED

Selected DME Specific Peptides for MIPEP Gene

Post-translational modifications for MIPEP Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for MIPEP Gene

ENSEMBL proteins:
REFSEQ proteins:

Domains for MIPEP Gene

Protein Domains for MIPEP Gene


  • Belongs to the peptidase M3 family.:
    • Q99797
genes like me logo Genes that share domains with MIPEP: view

No data available for Gene Families for MIPEP Gene

Function for MIPEP Gene

Molecular function for MIPEP Gene

GENATLAS Biochemistry: mitochondrial intermediate peptidase,involved in the biogenesis of the oxidative phosphorylation system
UniProtKB/Swiss-Prot CatalyticActivity: Release of an N-terminal octapeptide as second stage of processing of some proteins imported into the mitochondrion
UniProtKB/Swiss-Prot EnzymeRegulation: Activity is divalent cation-dependent. It is stimulated by manganese, magnesium or calcium ions and reversibly inhibited by zinc, cobalt and iron (By similarity).
UniProtKB/Swiss-Prot Function: Cleaves proteins, imported into the mitochondrion, to their mature size

Enzyme Numbers (IUBMB) for MIPEP Gene

Gene Ontology (GO) - Molecular Function for MIPEP Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004222 metalloendopeptidase activity IEA --
GO:0046872 metal ion binding IEA --
genes like me logo Genes that share ontologies with MIPEP: view

Phenotypes for MIPEP Gene

GenomeRNAi human phenotypes for MIPEP:
genes like me logo Genes that share phenotypes with MIPEP: view

miRNA for MIPEP Gene

miRTarBase miRNAs that target MIPEP

No data available for Animal Models , Transcription Factor Targeting and HOMER Transcription for MIPEP Gene

Localization for MIPEP Gene

Subcellular locations from UniProtKB/Swiss-Prot for MIPEP Gene

Mitochondrion matrix.

Subcellular locations from

Jensen Localization Image for MIPEP Gene COMPARTMENTS Subcellular localization image for MIPEP gene
Compartment Confidence
mitochondrion 5
cytosol 2
nucleus 1
peroxisome 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for MIPEP Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA --
GO:0005759 mitochondrial matrix IEA --
genes like me logo Genes that share ontologies with MIPEP: view

Pathways for MIPEP Gene

genes like me logo Genes that share pathways with MIPEP: view

Pathways by source for MIPEP Gene

Gene Ontology (GO) - Biological Process for MIPEP Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006508 proteolysis --
GO:0006627 protein processing involved in protein targeting to mitochondrion TAS 9073519
genes like me logo Genes that share ontologies with MIPEP: view

Compounds for MIPEP Gene

(5) HMDB Compounds for MIPEP Gene

Compound Synonyms Cas Number PubMed IDs
  • Ca
  • Co
  • Armco iron
  • Magnesium
  • manganese

(4) Novoseek inferred chemical compound relationships for MIPEP Gene

Compound -log(P) Hits PubMed IDs
ornithine 56.8 2
iron-sulfur 51.8 1
malate 44.6 2
iron 6.11 1
genes like me logo Genes that share compounds with MIPEP: view

Transcripts for MIPEP Gene

Unigene Clusters for MIPEP Gene

Mitochondrial intermediate peptidase:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for MIPEP Gene

ExUns: 1 ^ 2 ^ 3 ^ 4 ^ 5 ^ 6 ^ 7 ^ 8 ^ 9 ^ 10
SP3: -

Relevant External Links for MIPEP Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for MIPEP Gene

mRNA expression in normal human tissues for MIPEP Gene

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for MIPEP Gene

SOURCE GeneReport for Unigene cluster for MIPEP Gene Hs.507498

genes like me logo Genes that share expressions with MIPEP: view

Orthologs for MIPEP Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for MIPEP Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia MIPEP 36
  • 99.63 (n)
  • 99.44 (a)
  • 99 (a)
(Bos Taurus)
Mammalia MIPEP 36
  • 82.3 (n)
  • 89.35 (a)
  • 87 (a)
(Canis familiaris)
Mammalia MIPEP 36
  • 90.09 (n)
  • 92.26 (a)
  • 94 (a)
(Mus musculus)
Mammalia Mipep 36
  • 83.14 (n)
  • 85.57 (a)
Mipep 16
Mipep 37
  • 84 (a)
(Monodelphis domestica)
Mammalia MIPEP 37
  • 82 (a)
(Ornithorhynchus anatinus)
Mammalia MIPEP 37
  • 82 (a)
(Rattus norvegicus)
Mammalia Mipep 36
  • 82.71 (n)
  • 86.21 (a)
(Gallus gallus)
Aves MIPEP 36
  • 76.63 (n)
  • 80.84 (a)
  • 79 (a)
(Anolis carolinensis)
Reptilia MIPEP 37
  • 79 (a)
African clawed frog
(Xenopus laevis)
Amphibia Xl.23844 36
tropical clawed frog
(Silurana tropicalis)
Amphibia mipep 36
  • 72.31 (n)
  • 78.94 (a)
Str.10085 36
(Danio rerio)
Actinopterygii Dr.29606 36
mipep 36
  • 69 (n)
  • 73.62 (a)
mipep 37
  • 72 (a)
MIPEP (1 of 2) 37
  • 71 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP007728 36
  • 56.11 (n)
  • 51.2 (a)
fruit fly
(Drosophila melanogaster)
Insecta CG7791 36
  • 52.53 (n)
  • 50.38 (a)
CG7791 37
  • 48 (a)
CG7791 38
  • 48 (a)
(Caenorhabditis elegans)
Secernentea CELE_Y67H2A.7 36
  • 46.52 (n)
  • 36.11 (a)
Y67H2A.7 37
  • 33 (a)
Y67H2A.7 38
  • 39 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AFR198W 36
  • 47.09 (n)
  • 38.66 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes OCT1 36
  • 46.93 (n)
  • 34.88 (a)
OCT1 37
  • 30 (a)
OCT1 39
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0B12397g 36
  • 44.65 (n)
  • 35.65 (a)
thale cress
(Arabidopsis thaliana)
eudicotyledons AT5G51540 36
  • 47.83 (n)
  • 34.67 (a)
(Oryza sativa)
Liliopsida Os06g0686500 36
  • 46.42 (n)
  • 34.36 (a)
bread mold
(Neurospora crassa)
Ascomycetes NCU02063 36
  • 45.05 (n)
  • 36.41 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes oct1 36
  • 48.08 (n)
  • 37.12 (a)
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.1536 36
sea squirt
(Ciona savignyi)
Ascidiacea CSA.690 37
  • 49 (a)
Species with no ortholog for MIPEP:
  • Actinobacteria (Mycobacterium tuberculosis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for MIPEP Gene

Gene Tree for MIPEP (if available)
Gene Tree for MIPEP (if available)

Paralogs for MIPEP Gene Pseudogenes for MIPEP Gene

genes like me logo Genes that share paralogs with MIPEP: view

No data available for Paralogs for MIPEP Gene

Variants for MIPEP Gene

Sequence variations from dbSNP and Humsavar for MIPEP Gene

SNP ID Clin Chr 13 pos Sequence Context AA Info Type MAF
rs1108152 -- 23,738,307(-) gaaaa(A/C)acttc intron-variant
rs1011363 -- 23,803,046(+) AAATA(C/T)AAGAT intron-variant
rs1011362 -- 23,803,018(+) gcatc(A/G)taagt intron-variant
rs943065 -- 23,771,423(-) GTAAA(C/T)TGTTT intron-variant
rs943064 -- 23,779,264(-) acatc(A/G)ctcac intron-variant

Structural Variations from Database of Genomic Variants (DGV) for MIPEP Gene

Variant ID Type Subtype PubMed ID
esv2422502 CNV Duplication 17116639
esv2747122 CNV Deletion 23290073
esv5325 CNV Complex 18987735
nsv455837 CNV Gain 19166990
nsv525965 CNV Gain 19592680
dgv1561n71 CNV Gain 21882294
esv2751136 CNV Loss 17911159
dgv1565n71 CNV Gain 21882294
esv2747134 CNV Deletion 23290073
esv2659554 CNV Deletion 23128226
nsv899927 CNV Gain 21882294
nsv899928 CNV Loss 21882294
esv21543 CNV Gain 19812545
nsv832566 CNV Gain 17160897
esv2658569 CNV Deletion 23128226

Relevant External Links for MIPEP Gene

HapMap Linkage Disequilibrium report

Disorders for MIPEP Gene

(2) Novoseek inferred disease relationships for MIPEP Gene

Disease -log(P) Hits PubMed IDs
friedreich ataxia 60.8 1
neurodegenerative diseases 21.2 2

Relevant External Links for MIPEP

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with MIPEP: view

No data available for UniProtKB/Swiss-Prot for MIPEP Gene

Publications for MIPEP Gene

  1. Cloning, expression, and chromosomal assignment of the human mitochondrial intermediate peptidase gene (MIPEP). (PMID: 9073519) Chew A. … Isaya G. (Genomics 1997) 2 3 4 23
  2. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PMID: 20877624) Hendrickson S.L. … O'Brien S.J. (PLoS ONE 2010) 3 49
  3. Functional and genomic analysis of the human mitochondrial intermediate peptidase, a putative protein partner of frataxin. (PMID: 10783257) Chew A. … Isaya G. (Genomics 2000) 3 23
  4. Genetic variants at 13q12.12 are associated with high myopia in the Han Chinese population. (PMID: 21640322) Shi Y. … Yang Z. (Am. J. Hum. Genet. 2011) 3 49
  5. A genome-wide association study identifies two new lung cancer susceptibility loci at 13q12.12 and 22q12.2 in Han Chinese. (PMID: 21725308) Hu Z. … Shen H. (Nat. Genet. 2011) 3

Products for MIPEP Gene

Sources for MIPEP Gene

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