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Protein

Mitochondria-eating protein

Gene

SPATA18

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

Functioni

Key regulator of mitochondrial quality that mediates the repairing or degradation of unhealthy mitochondria in response to mitochondrial damage. Mediator of mitochondrial protein catabolic process (also named MALM) by mediating the degradation of damaged proteins inside mitochondria by promoting the accumulation in the mitochondrial matrix of hydrolases that are characteristic of the lysosomal lumen. Also involved in mitochondrion degradation of damaged mitochondria by promoting the formation of vacuole-like structures (named MIV), which engulf and degrade unhealthy mitochondria by accumulating lysosomes. The physical interaction of SPATA18/MIEAP, BNIP3 and BNIP3L/NIX at the mitochondrial outer membrane regulates the opening of a pore in the mitochondrial double membrane in order to mediate the translocation of lysosomal proteins from the cytoplasm to the mitochondrial matrix.3 Publications

GO - Biological processi

  • cellular response to DNA damage stimulus Source: UniProtKB
  • mitochondrial protein catabolic process Source: UniProtKB
  • mitophagy by induced vacuole formation Source: UniProtKB
Complete GO annotation...

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondria-eating protein
Alternative name(s):
Spermatogenesis-associated protein 18
Gene namesi
Name:SPATA18
Synonyms:MIEAP
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

HGNCiHGNC:29579. SPATA18.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: UniProtKB
  • intracellular membrane-bounded organelle Source: UniProtKB
  • mitochondrial outer membrane Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Membrane, Mitochondrion, Mitochondrion outer membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA142670883.

Polymorphism and mutation databases

BioMutaiSPATA18.
DMDMi74751399.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 538538Mitochondria-eating proteinPRO_0000254165Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei85 – 851PhosphoserineBy similarity
Modified residuei156 – 1561PhosphoserineBy similarity
Modified residuei159 – 1591PhosphoserineBy similarity
Modified residuei287 – 2871PhosphoserineBy similarity
Modified residuei509 – 5091PhosphoserineBy similarity

Keywords - PTMi

Phosphoprotein

Proteomic databases

PaxDbiQ8TC71.
PRIDEiQ8TC71.

PTM databases

iPTMnetiQ8TC71.
PhosphoSiteiQ8TC71.

Expressioni

Inductioni

By p53/TP53 and p63/TP63. Directly activated by p53/TP53.2 Publications

Gene expression databases

BgeeiQ8TC71.
CleanExiHS_SPATA18.
ExpressionAtlasiQ8TC71. baseline and differential.
GenevisibleiQ8TC71. HS.

Organism-specific databases

HPAiHPA036854.

Interactioni

Subunit structurei

Interacts (via coiled-coil domains) with BNIP3L (via BH3 domain). Interacts (via coiled-coil domains) with BNIP3 (via BH3 domain).2 Publications

Protein-protein interaction databases

BioGridi126329. 3 interactions.
IntActiQ8TC71. 2 interactions.
STRINGi9606.ENSP00000295213.

Structurei

3D structure databases

ProteinModelPortaliQ8TC71.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Coiled coil

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Coiled coili118 – 18770Sequence analysisAdd
BLAST
Coiled coili219 – 25638Sequence analysisAdd
BLAST

Compositional bias

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Compositional biasi257 – 31357Ser-richAdd
BLAST

Sequence similaritiesi

Belongs to the MIEAP family.Curated

Keywords - Domaini

Coiled coil

Phylogenomic databases

eggNOGiENOG410IGBW. Eukaryota.
ENOG410XRYY. LUCA.
GeneTreeiENSGT00390000013532.
HOGENOMiHOG000070232.
HOVERGENiHBG093990.
InParanoidiQ8TC71.
OMAiNTCDQNL.
OrthoDBiEOG7CG702.
PhylomeDBiQ8TC71.
TreeFamiTF328808.

Family and domain databases

InterProiIPR026169. MIEAP.
IPR031981. MIEAP_C.
[Graphical view]
PANTHERiPTHR21771. PTHR21771. 1 hit.
PfamiPF16026. MIEAP. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q8TC71-1) [UniParc]FASTAAdd to basket

Also known as: Alpha, Mieap-alpha

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAENLKRLVS NETLRTLQEK LDFWLKEYNT NTCDQNLNHC LELIEQVAKV
60 70 80 90 100
QGQLFGILTA AAQEGGRNDG VETIKSRLLP WLEASFTAAS LGKSVDSKVP
110 120 130 140 150
SLQDTFDRER HKDPSPRDRD MQQLDSNLNS TRSQCNQVQD DLVETEKNLE
160 170 180 190 200
ESKNRSAISL LAAEEEINQL KKQLKSLQAQ EDARHRNTDQ RSSENRRSEP
210 220 230 240 250
WSLEERKREQ WNSLKQNADQ QDTEAMSDYK KQLRNLKEEI AVLSAEKSAL
260 270 280 290 300
QGRSSRSRSP SPAPRSRSCS RSRSASPSTA VKVRRPSPNR SKLSNVARKA
310 320 330 340 350
ALLSRFSDSY SQARLDAQCL LRRCIDKAET VQRIIYIATV EAFHVAKMAF
360 370 380 390 400
RHFKIHVRKS LTPSYVGSND FENAVLDYVI CHLDLYDSQS SVNDVIRAMN
410 420 430 440 450
VNPKISFPPV VDFCLLSDFI QEICCIAFAM QALEPPLDIA YGADGEVFND
460 470 480 490 500
CKYRRSYDSD FTAPLVLYHV WPALMENDCV IMKGEAVTRR GAFWNSVRSV
510 520 530
SRCRSRSLSP ICPRSQIGLN TMSRSRSPSP IRCGLPRF
Length:538
Mass (Da):61,109
Last modified:June 1, 2002 - v1
Checksum:i365754F577F8ED7F
GO
Isoform 2 (identifier: Q8TC71-2) [UniParc]FASTAAdd to basket

Also known as: Beta, Mieap-beta

The sequence of this isoform differs from the canonical sequence as follows:
     141-173: DLVETEKNLEESKNRSAISLLAAEEEINQLKKQ → E

Show »
Length:506
Mass (Da):57,485
Checksum:i5ACADC54EF2F5A0D
GO

Sequence cautioni

The sequence BAC05356.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti318 – 3181Q → E in AAH37886 (PubMed:15489334).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti227 – 2271S → P.1 Publication
Corresponds to variant rs3860707 [ dbSNP | Ensembl ].
VAR_028828
Natural varianti483 – 4831K → R.1 Publication
Corresponds to variant rs11558773 [ dbSNP | Ensembl ].
VAR_028829

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei141 – 17333DLVET…QLKKQ → E in isoform 2. 2 PublicationsVSP_041048Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB465501 mRNA. Translation: BAJ53738.1.
AB465502 mRNA. Translation: BAJ53739.1.
AK098625 mRNA. Translation: BAC05356.1. Different initiation.
AK304386 mRNA. Translation: BAG65222.1.
AC093858 Genomic DNA. No translation available.
CH471057 Genomic DNA. Translation: EAX05433.1.
BC025396 mRNA. Translation: AAH25396.1.
BC037886 mRNA. Translation: AAH37886.1.
CCDSiCCDS3489.1. [Q8TC71-1]
CCDS75124.1. [Q8TC71-2]
RefSeqiNP_001284537.1. NM_001297608.1. [Q8TC71-2]
NP_660306.1. NM_145263.3. [Q8TC71-1]
UniGeneiHs.527090.

Genome annotation databases

EnsembliENST00000295213; ENSP00000295213; ENSG00000163071. [Q8TC71-1]
ENST00000419395; ENSP00000415309; ENSG00000163071. [Q8TC71-2]
GeneIDi132671.
KEGGihsa:132671.
UCSCiuc003gzl.4. human. [Q8TC71-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB465501 mRNA. Translation: BAJ53738.1.
AB465502 mRNA. Translation: BAJ53739.1.
AK098625 mRNA. Translation: BAC05356.1. Different initiation.
AK304386 mRNA. Translation: BAG65222.1.
AC093858 Genomic DNA. No translation available.
CH471057 Genomic DNA. Translation: EAX05433.1.
BC025396 mRNA. Translation: AAH25396.1.
BC037886 mRNA. Translation: AAH37886.1.
CCDSiCCDS3489.1. [Q8TC71-1]
CCDS75124.1. [Q8TC71-2]
RefSeqiNP_001284537.1. NM_001297608.1. [Q8TC71-2]
NP_660306.1. NM_145263.3. [Q8TC71-1]
UniGeneiHs.527090.

3D structure databases

ProteinModelPortaliQ8TC71.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi126329. 3 interactions.
IntActiQ8TC71. 2 interactions.
STRINGi9606.ENSP00000295213.

PTM databases

iPTMnetiQ8TC71.
PhosphoSiteiQ8TC71.

Polymorphism and mutation databases

BioMutaiSPATA18.
DMDMi74751399.

Proteomic databases

PaxDbiQ8TC71.
PRIDEiQ8TC71.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000295213; ENSP00000295213; ENSG00000163071. [Q8TC71-1]
ENST00000419395; ENSP00000415309; ENSG00000163071. [Q8TC71-2]
GeneIDi132671.
KEGGihsa:132671.
UCSCiuc003gzl.4. human. [Q8TC71-1]

Organism-specific databases

CTDi132671.
GeneCardsiSPATA18.
HGNCiHGNC:29579. SPATA18.
HPAiHPA036854.
MIMi612814. gene.
neXtProtiNX_Q8TC71.
PharmGKBiPA142670883.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IGBW. Eukaryota.
ENOG410XRYY. LUCA.
GeneTreeiENSGT00390000013532.
HOGENOMiHOG000070232.
HOVERGENiHBG093990.
InParanoidiQ8TC71.
OMAiNTCDQNL.
OrthoDBiEOG7CG702.
PhylomeDBiQ8TC71.
TreeFamiTF328808.

Miscellaneous databases

GenomeRNAii132671.
NextBioi83118.
PROiQ8TC71.
SOURCEiSearch...

Gene expression databases

BgeeiQ8TC71.
CleanExiHS_SPATA18.
ExpressionAtlasiQ8TC71. baseline and differential.
GenevisibleiQ8TC71. HS.

Family and domain databases

InterProiIPR026169. MIEAP.
IPR031981. MIEAP_C.
[Graphical view]
PANTHERiPTHR21771. PTHR21771. 1 hit.
PfamiPF16026. MIEAP. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Possible existence of lysosome-like organella within mitochondria and its role in mitochondrial quality control."
    Miyamoto Y., Kitamura N., Nakamura Y., Futamura M., Miyamoto T., Yoshida M., Ono M., Ichinose S., Arakawa H.
    PLoS ONE 6:E16054-E16054(2011) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS 1 AND 2), FUNCTION, SUBCELLULAR LOCATION, INDUCTION.
  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] (ISOFORM 2), NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 135-538 (ISOFORM 1), VARIANT PRO-227.
    Tissue: Testis and Trachea.
  3. "Generation and annotation of the DNA sequences of human chromosomes 2 and 4."
    Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H.
    , Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K.
    Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  4. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  5. "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] (ISOFORM 1), VARIANT ARG-483.
  6. "SPATA18, a spermatogenesis-associated gene, is a novel transcriptional target of p53 and p63."
    Bornstein C., Brosh R., Molchadsky A., Madar S., Kogan-Sakin I., Goldstein I., Chakravarti D., Flores E.R., Goldfinger N., Sarig R., Rotter V.
    Mol. Cell. Biol. 31:1679-1689(2011) [PubMed] [Europe PMC] [Abstract]
    Cited for: INDUCTION.
  7. "Mieap, a p53-inducible protein, controls mitochondrial quality by repairing or eliminating unhealthy mitochondria."
    Kitamura N., Nakamura Y., Miyamoto Y., Miyamoto T., Kabu K., Yoshida M., Futamura M., Ichinose S., Arakawa H.
    PLoS ONE 6:E16060-E16060(2011) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, SUBCELLULAR LOCATION, INTERACTION WITH BNIP3L.
  8. "BNIP3 and NIX mediate Mieap-induced accumulation of lysosomal proteins within mitochondria."
    Nakamura Y., Kitamura N., Shinogi D., Yoshida M., Goda O., Murai R., Kamino H., Arakawa H.
    PLoS ONE 7:E30767-E30767(2012) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, SUBCELLULAR LOCATION, INTERACTION WITH BNIP3 AND BNIP3L.

Entry informationi

Entry nameiMIEAP_HUMAN
AccessioniPrimary (citable) accession number: Q8TC71
Secondary accession number(s): B4E2R0
, E5RLK1, Q8IY48, Q8N7D7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 31, 2006
Last sequence update: June 1, 2002
Last modified: March 16, 2016
This is version 100 of the entry and version 1 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 4
    Human chromosome 4: 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. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.