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Q99797

- MIPEP_HUMAN

UniProt

Q99797 - MIPEP_HUMAN

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Protein

Mitochondrial intermediate peptidase

Gene
MIPEP, MIP
Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

Functioni

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

Catalytic activityi

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

Cofactori

Binds 1 zinc ion By similarity.

Enzyme regulationi

Activity is divalent cation-dependent. It is stimulated by manganese, magnesium or calcium ions and reversibly inhibited by zinc, cobalt and iron By similarity.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi495 – 4951Zinc; catalytic By similarity
Active sitei496 – 4961 By similarity
Metal bindingi499 – 4991Zinc; catalytic By similarity
Metal bindingi502 – 5021Zinc; catalytic By similarity

GO - Molecular functioni

  1. metal ion binding Source: UniProtKB-KW
  2. metalloendopeptidase activity Source: ProtInc

GO - Biological processi

  1. protein processing involved in protein targeting to mitochondrion Source: ProtInc
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

Calcium, Cobalt, Iron, Magnesium, Manganese, Metal-binding, Zinc

Protein family/group databases

MEROPSiM03.A05.

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial intermediate peptidase (EC:3.4.24.59)
Short name:
MIP
Gene namesi
Name:MIPEP
Synonyms:MIP
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 13

Organism-specific databases

HGNCiHGNC:7104. MIPEP.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial matrix Source: UniProtKB-SubCell
  2. mitochondrion Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA30822.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3535MitochondrionAdd
BLAST
Chaini36 – 713678Mitochondrial intermediate peptidasePRO_0000028579Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei126 – 1261N6-acetyllysine1 Publication

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiQ99797.
PaxDbiQ99797.
PRIDEiQ99797.

PTM databases

PhosphoSiteiQ99797.

Expressioni

Gene expression databases

ArrayExpressiQ99797.
BgeeiQ99797.
CleanExiHS_MIP.
HS_MIPEP.
GenevestigatoriQ99797.

Organism-specific databases

HPAiHPA030676.
HPA031669.
HPA031670.

Interactioni

Subunit structurei

Monomer By similarity.

Protein-protein interaction databases

BioGridi110431. 5 interactions.
IntActiQ99797. 4 interactions.
MINTiMINT-2815808.
STRINGi9606.ENSP00000371607.

Structurei

3D structure databases

ProteinModelPortaliQ99797.
SMRiQ99797. Positions 120-694.

Family & Domainsi

Sequence similaritiesi

Belongs to the peptidase M3 family.

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0339.
HOGENOMiHOG000230535.
HOVERGENiHBG008215.
InParanoidiQ99797.
KOiK01410.
OMAiLQVFYSA.
OrthoDBiEOG7TTQ72.
PhylomeDBiQ99797.
TreeFamiTF105715.

Family and domain databases

Gene3Di1.10.1370.10. 2 hits.
1.20.1050.40. 1 hit.
3.40.390.10. 1 hit.
InterProiIPR024079. MetalloPept_cat_dom.
IPR024077. Neurolysin/TOP_dom2.
IPR024080. Neurolysin/TOP_N.
IPR001567. Pept_M3A_M3B.
[Graphical view]
PfamiPF01432. Peptidase_M3. 1 hit.
[Graphical view]
PROSITEiPS00142. ZINC_PROTEASE. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q99797-1 [UniParc]FASTAAdd to Basket

« Hide

MLCVGRLGGL GARAAALPPR RAGRGSLEAG IRARRVSTSW SPVGAAFNVK    50
PQGSRLDLFG ERRGLFGVPE LSAPEGFHIA QEKALRKTEL LVDRACSTPP 100
GPQTVLIFDE LSDSLCRVAD LADFVKIAHP EPAFREAAEE ACRSIGTMVE 150
KLNTNVDLYQ SLQKLLADKK LVDSLDPETR RVAELFMFDF EISGIHLDKE 200
KRKRAVDLNV KILDLSSTFL MGTNFPNKIE KHLLPEHIRR NFTSAGDHII 250
IDGLHAESPD DLVREAAYKI FLYPNAGQLK CLEELLSSRD LLAKLVGYST 300
FSHRALQGTI AKNPETVMQF LEKLSDKLSE RTLKDFEMIR GMKMKLNPQN 350
SEVMPWDPPY YSGVIRAERY NIEPSLYCPF FSLGACMEGL NILLNRLLGI 400
SLYAEQPAKG EVWSEDVRKL AVVHESEGLL GYIYCDFFQR ADKPHQDCHF 450
TIRGGRLKED GDYQLPVVVL MLNLPRSSRS SPTLLTPSMM ENLFHEMGHA 500
MHSMLGRTRY QHVTGTRCPT DFAEVPSILM EYFANDYRVV NQFARHYQTG 550
QPLPKNMVSR LCESKKVCAA ADMQLQVFYA TLDQIYHGKH PLRNSTTDIL 600
KETQEKFYGL PYVPNTAWQL RFSHLVGYGA RYYSYLMSRA VASMVWKECF 650
LQDPFNRAAG ERYRREMLAH GGGREPMLMV EGMLQKCPSV DDFVSALVSD 700
LDLDFETFLM DSE 713
Length:713
Mass (Da):80,641
Last modified:February 26, 2008 - v2
Checksum:i9DBB26B74355B9C1
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti137 – 1371A → V.
Corresponds to variant rs2312296 [ dbSNP | Ensembl ].
VAR_038934
Natural varianti340 – 3401R → Q.
Corresponds to variant rs11551114 [ dbSNP | Ensembl ].
VAR_038935
Natural varianti453 – 4531R → H.
Corresponds to variant rs12858248 [ dbSNP | Ensembl ].
VAR_038936
Natural varianti488 – 4881S → G.3 Publications
Corresponds to variant rs7333040 [ dbSNP | Ensembl ].
VAR_038937

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti63 – 642RG → AR in AAC51231. 1 Publication
Sequence conflicti200 – 2001E → Q in AAC51231. 1 Publication
Sequence conflicti348 – 3481P → A in AAC51231. 1 Publication
Sequence conflicti467 – 4671V → L in AAC51231. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
U80034 mRNA. Translation: AAC51231.1.
AK291923 mRNA. Translation: BAF84612.1.
AL157368, AL139080, AL445985 Genomic DNA. Translation: CAI15091.1.
AL139080, AL157368, AL445985 Genomic DNA. Translation: CAI39630.1.
AL445985, AL139080, AL157368 Genomic DNA. Translation: CAM20040.1.
BC009934 mRNA. Translation: AAH09934.1.
CCDSiCCDS9303.1.
RefSeqiNP_005923.2. NM_005932.3.
UniGeneiHs.507498.

Genome annotation databases

EnsembliENST00000382172; ENSP00000371607; ENSG00000027001.
GeneIDi4285.
KEGGihsa:4285.
UCSCiuc001uox.4. human.

Polymorphism databases

DMDMi182639267.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
U80034 mRNA. Translation: AAC51231.1 .
AK291923 mRNA. Translation: BAF84612.1 .
AL157368 , AL139080 , AL445985 Genomic DNA. Translation: CAI15091.1 .
AL139080 , AL157368 , AL445985 Genomic DNA. Translation: CAI39630.1 .
AL445985 , AL139080 , AL157368 Genomic DNA. Translation: CAM20040.1 .
BC009934 mRNA. Translation: AAH09934.1 .
CCDSi CCDS9303.1.
RefSeqi NP_005923.2. NM_005932.3.
UniGenei Hs.507498.

3D structure databases

ProteinModelPortali Q99797.
SMRi Q99797. Positions 120-694.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 110431. 5 interactions.
IntActi Q99797. 4 interactions.
MINTi MINT-2815808.
STRINGi 9606.ENSP00000371607.

Protein family/group databases

MEROPSi M03.A05.

PTM databases

PhosphoSitei Q99797.

Polymorphism databases

DMDMi 182639267.

Proteomic databases

MaxQBi Q99797.
PaxDbi Q99797.
PRIDEi Q99797.

Protocols and materials databases

DNASUi 4285.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000382172 ; ENSP00000371607 ; ENSG00000027001 .
GeneIDi 4285.
KEGGi hsa:4285.
UCSCi uc001uox.4. human.

Organism-specific databases

CTDi 4285.
GeneCardsi GC13M024304.
HGNCi HGNC:7104. MIPEP.
HPAi HPA030676.
HPA031669.
HPA031670.
MIMi 602241. gene.
neXtProti NX_Q99797.
PharmGKBi PA30822.
GenAtlasi Search...

Phylogenomic databases

eggNOGi COG0339.
HOGENOMi HOG000230535.
HOVERGENi HBG008215.
InParanoidi Q99797.
KOi K01410.
OMAi LQVFYSA.
OrthoDBi EOG7TTQ72.
PhylomeDBi Q99797.
TreeFami TF105715.

Miscellaneous databases

ChiTaRSi MIPEP. human.
GenomeRNAii 4285.
NextBioi 16857.
PROi Q99797.
SOURCEi Search...

Gene expression databases

ArrayExpressi Q99797.
Bgeei Q99797.
CleanExi HS_MIP.
HS_MIPEP.
Genevestigatori Q99797.

Family and domain databases

Gene3Di 1.10.1370.10. 2 hits.
1.20.1050.40. 1 hit.
3.40.390.10. 1 hit.
InterProi IPR024079. MetalloPept_cat_dom.
IPR024077. Neurolysin/TOP_dom2.
IPR024080. Neurolysin/TOP_N.
IPR001567. Pept_M3A_M3B.
[Graphical view ]
Pfami PF01432. Peptidase_M3. 1 hit.
[Graphical view ]
PROSITEi PS00142. ZINC_PROTEASE. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning, expression, and chromosomal assignment of the human mitochondrial intermediate peptidase gene (MIPEP)."
    Chew A., Buck E.A., Peretz S., Sirugo G., Rinaldo P., Isaya G.
    Genomics 40:493-496(1997) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT GLY-488.
    Tissue: Liver.
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
    , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT GLY-488.
  3. "The DNA sequence and analysis of human chromosome 13."
    Dunham A., Matthews L.H., Burton J., Ashurst J.L., Howe K.L., Ashcroft K.J., Beare D.M., Burford D.C., Hunt S.E., Griffiths-Jones S., Jones M.C., Keenan S.J., Oliver K., Scott C.E., Ainscough R., Almeida J.P., Ambrose K.D., Andrews D.T.
    , Ashwell R.I.S., Babbage A.K., Bagguley C.L., Bailey J., Bannerjee R., Barlow K.F., Bates K., Beasley H., Bird C.P., Bray-Allen S., Brown A.J., Brown J.Y., Burrill W., Carder C., Carter N.P., Chapman J.C., Clamp M.E., Clark S.Y., Clarke G., Clee C.M., Clegg S.C., Cobley V., Collins J.E., Corby N., Coville G.J., Deloukas P., Dhami P., Dunham I., Dunn M., Earthrowl M.E., Ellington A.G., Faulkner L., Frankish A.G., Frankland J., French L., Garner P., Garnett J., Gilbert J.G.R., Gilson C.J., Ghori J., Grafham D.V., Gribble S.M., Griffiths C., Hall R.E., Hammond S., Harley J.L., Hart E.A., Heath P.D., Howden P.J., Huckle E.J., Hunt P.J., Hunt A.R., Johnson C., Johnson D., Kay M., Kimberley A.M., King A., Laird G.K., Langford C.J., Lawlor S., Leongamornlert D.A., Lloyd D.M., Lloyd C., Loveland J.E., Lovell J., Martin S., Mashreghi-Mohammadi M., McLaren S.J., McMurray A., Milne S., Moore M.J.F., Nickerson T., Palmer S.A., Pearce A.V., Peck A.I., Pelan S., Phillimore B., Porter K.M., Rice C.M., Searle S., Sehra H.K., Shownkeen R., Skuce C.D., Smith M., Steward C.A., Sycamore N., Tester J., Thomas D.W., Tracey A., Tromans A., Tubby B., Wall M., Wallis J.M., West A.P., Whitehead S.L., Willey D.L., Wilming L., Wray P.W., Wright M.W., Young L., Coulson A., Durbin R.M., Hubbard T., Sulston J.E., Beck S., Bentley D.R., Rogers J., Ross M.T.
    Nature 428:522-528(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT GLY-488.
    Tissue: Uterus.
  5. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
    Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
    Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-126, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  6. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiMIPEP_HUMAN
AccessioniPrimary (citable) accession number: Q99797
Secondary accession number(s): Q5JV15, Q5T9Q9, Q96G65
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: February 26, 2008
Last modified: July 9, 2014
This is version 125 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 13
    Human chromosome 13: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. Peptidase families
    Classification of peptidase families and list of entries
  6. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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