MTX1_MOUSE
ID MTX1_MOUSE Reviewed; 317 AA.
AC P47802;
DT 01-FEB-1996, integrated into UniProtKB/Swiss-Prot.
DT 01-FEB-1996, sequence version 1.
DT 25-MAY-2022, entry version 138.
DE RecName: Full=Metaxin-1;
DE AltName: Full=Mitochondrial outer membrane import complex protein 1;
GN Name=Mtx1; Synonyms=Mtx, Mtxn;
OS Mus musculus (Mouse).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae;
OC Murinae; Mus; Mus.
OX NCBI_TaxID=10090;
RN [1]
RP NUCLEOTIDE SEQUENCE [MRNA].
RC STRAIN=C57BL/6J; TISSUE=Brain;
RX PubMed=7753840; DOI=10.1073/pnas.92.10.4547;
RA Bornstein P., McKinney C.E., Lamarca M.E., Winfield S., Shingu T.,
RA Devarayalu S., Vos H.L., Ginns E.I.;
RT "Metaxin, a gene contiguous to both thrombospondin 3 and
RT glucocerebrosidase, is required for embryonic development in the mouse:
RT implications for Gaucher disease.";
RL Proc. Natl. Acad. Sci. U.S.A. 92:4547-4551(1995).
RN [2]
RP NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-27.
RX PubMed=8871542; DOI=10.1093/nar/24.19.3661;
RA Collins M., Bornstein P.;
RT "SP1-binding elements, within the common metaxin-thrombospondin 3
RT intergenic region, participate in the regulation of the metaxin gene.";
RL Nucleic Acids Res. 24:3661-3669(1996).
RN [3]
RP PROTEIN SEQUENCE OF 89-103; 118-124 AND 169-175, AND IDENTIFICATION BY MASS
RP SPECTROMETRY.
RC STRAIN=C57BL/6J; TISSUE=Brain;
RA Lubec G., Kang S.U.;
RL Submitted (APR-2007) to UniProtKB.
RN [4]
RP NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 109-317.
RX PubMed=9705320; DOI=10.1074/jbc.273.34.21816;
RA Collins M., Rojnuckarin P., Zhu Y.H., Bornstein P.;
RT "A far upstream, cell type-specific enhancer of the mouse thrombospondin 3
RT gene is located within intron 6 of the adjacent metaxin gene.";
RL J. Biol. Chem. 273:21816-21824(1998).
RN [5]
RP FUNCTION, AND SUBCELLULAR LOCATION.
RX PubMed=9045676; DOI=10.1074/jbc.272.10.6510;
RA Armstrong L.C., Komiya T., Bergman B.E., Mihara K., Bornstein P.;
RT "Metaxin is a component of a preprotein import complex in the outer
RT membrane of the mammalian mitochondrion.";
RL J. Biol. Chem. 272:6510-6518(1997).
RN [6]
RP INTERACTION WITH MTX2.
RX PubMed=10381257;
RX DOI=10.1002/(sici)1097-4644(19990701)74:1<11::aid-jcb2>3.0.co;2-v;
RA Armstrong L.C., Saenz A.J., Bornstein P.;
RT "Metaxin 1 interacts with metaxin 2, a novel related protein associated
RT with the mammalian mitochondrial outer membrane.";
RL J. Cell. Biochem. 74:11-22(1999).
RN [7]
RP IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
RC TISSUE=Brain, Brown adipose tissue, Heart, Kidney, Liver, Lung, Spleen, and
RC Testis;
RX PubMed=21183079; DOI=10.1016/j.cell.2010.12.001;
RA Huttlin E.L., Jedrychowski M.P., Elias J.E., Goswami T., Rad R.,
RA Beausoleil S.A., Villen J., Haas W., Sowa M.E., Gygi S.P.;
RT "A tissue-specific atlas of mouse protein phosphorylation and expression.";
RL Cell 143:1174-1189(2010).
CC -!- FUNCTION: Involved in transport of proteins into the mitochondrion.
CC Essential for embryonic development. {ECO:0000269|PubMed:9045676}.
CC -!- SUBUNIT: Interacts with MTX2/metaxin-2 (PubMed:10381257). Associates
CC with the mitochondrial contact site and cristae organizing system
CC (MICOS) complex, composed of at least MICOS10/MIC10, CHCHD3/MIC19,
CC CHCHD6/MIC25, APOOL/MIC27, IMMT/MIC60, APOO/MIC23/MIC26 and QIL1/MIC13
CC (By similarity). This complex was also known under the names MINOS or
CC MitOS complex (By similarity). The MICOS complex associates with
CC mitochondrial outer membrane proteins SAMM50, MTX1 and MTX2 (together
CC described as components of the mitochondrial outer membrane sorting
CC assembly machinery (SAM) complex) and DNAJC11, mitochondrial inner
CC membrane protein TMEM11 and with HSPA9 (By similarity). The MICOS and
CC SAM complexes together with DNAJC11 are part of a large protein complex
CC spanning both membranes termed the mitochondrial intermembrane space
CC bridging (MIB) complex (By similarity). Interacts with ARMC1 (By
CC similarity). {ECO:0000250|UniProtKB:Q13505,
CC ECO:0000269|PubMed:10381257}.
CC -!- SUBCELLULAR LOCATION: Mitochondrion outer membrane
CC {ECO:0000269|PubMed:9045676}.
CC -!- TISSUE SPECIFICITY: Ubiquitous. Higher levels are seen in the kidney as
CC compared to other tissues.
CC -!- PTM: Ubiquitinated by PRKN during mitophagy, leading to its degradation
CC and enhancement of mitophagy. Deubiquitinated by USP30.
CC {ECO:0000250|UniProtKB:Q13505}.
CC -!- SIMILARITY: Belongs to the metaxin family. {ECO:0000305}.
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DR EMBL; L36962; AAC37672.1; -; mRNA.
DR EMBL; U66257; AAC52818.1; -; Genomic_DNA.
DR EMBL; AF059277; AAC63229.1; -; Genomic_DNA.
DR CCDS; CCDS17494.2; -.
DR PIR; I59379; I59379.
DR AlphaFoldDB; P47802; -.
DR SMR; P47802; -.
DR CORUM; P47802; -.
DR IntAct; P47802; 1.
DR STRING; 10090.ENSMUSP00000073261; -.
DR iPTMnet; P47802; -.
DR PhosphoSitePlus; P47802; -.
DR SwissPalm; P47802; -.
DR EPD; P47802; -.
DR jPOST; P47802; -.
DR MaxQB; P47802; -.
DR PaxDb; P47802; -.
DR PeptideAtlas; P47802; -.
DR PRIDE; P47802; -.
DR ProteomicsDB; 290123; -.
DR MGI; MGI:103025; Mtx1.
DR eggNOG; KOG3028; Eukaryota.
DR InParanoid; P47802; -.
DR PhylomeDB; P47802; -.
DR Reactome; R-MMU-9013404; RAC2 GTPase cycle.
DR PRO; PR:P47802; -.
DR Proteomes; UP000000589; Unplaced.
DR RNAct; P47802; protein.
DR GO; GO:0005737; C:cytoplasm; IBA:GO_Central.
DR GO; GO:0016021; C:integral component of membrane; IEA:UniProtKB-KW.
DR GO; GO:0005743; C:mitochondrial inner membrane; HDA:MGI.
DR GO; GO:0005739; C:mitochondrion; HDA:MGI.
DR GO; GO:0001401; C:SAM complex; ISO:MGI.
DR GO; GO:0007005; P:mitochondrion organization; IBA:GO_Central.
DR GO; GO:0015031; P:protein transport; IEA:UniProtKB-KW.
DR InterPro; IPR036282; Glutathione-S-Trfase_C_sf.
DR InterPro; IPR040079; Glutathione_S-Trfase.
DR InterPro; IPR017410; Metaxin1/3.
DR InterPro; IPR033468; Metaxin_GST.
DR InterPro; IPR019564; Sam37/metaxin_N.
DR Pfam; PF17171; GST_C_6; 1.
DR Pfam; PF10568; Tom37; 1.
DR PIRSF; PIRSF038150; Metaxin; 1.
DR SFLD; SFLDS00019; Glutathione_Transferase_(cytos; 1.
DR SUPFAM; SSF47616; SSF47616; 1.
PE 1: Evidence at protein level;
KW Direct protein sequencing; Isopeptide bond; Membrane; Mitochondrion;
KW Mitochondrion outer membrane; Protein transport; Reference proteome;
KW Transmembrane; Transmembrane helix; Transport; Ubl conjugation.
FT CHAIN 1..317
FT /note="Metaxin-1"
FT /id="PRO_0000220992"
FT TRANSMEM 272..292
FT /note="Helical"
FT /evidence="ECO:0000255"
FT CROSSLNK 38
FT /note="Glycyl lysine isopeptide (Lys-Gly) (interchain with
FT G-Cter in ubiquitin)"
FT /evidence="ECO:0000250|UniProtKB:Q13505"
FT CROSSLNK 41
FT /note="Glycyl lysine isopeptide (Lys-Gly) (interchain with
FT G-Cter in ubiquitin)"
FT /evidence="ECO:0000250|UniProtKB:Q13505"
FT CROSSLNK 78
FT /note="Glycyl lysine isopeptide (Lys-Gly) (interchain with
FT G-Cter in ubiquitin)"
FT /evidence="ECO:0000250|UniProtKB:Q13505"
FT CROSSLNK 168
FT /note="Glycyl lysine isopeptide (Lys-Gly) (interchain with
FT G-Cter in ubiquitin)"
FT /evidence="ECO:0000250|UniProtKB:Q13505"
SQ SEQUENCE 317 AA; 35624 MW; F7528371DC986562 CRC64;
MAAPMELFCW SGGWGLPSVD LDSLAVLTYT RFTGAPLKIH KTSNPWQSPS GTLPALRTSD
GKVITVPDKI ITHLRKEKYN ADYDLSARQG ADTLAFMSLL EEKLLPVLIH TFWIDAKNYV
EVTRKWYAEA MPFPLNFFLP GRMQRQYMER LQLLCGEHKS ENEEELEKEL YQEARECLTL
LSQRLGSQKF FFGDAPASLD AFVFSHLALL LQAKLPSGKL QAHLRGLHNL CAYCTHILNL
YFPRDGDEVP LPRQTPAAPE TEEEPYRRRT QILSVLAGLA AMVGYALLSG IVSIQRTSPA
RAPGTRALGL AEEDEED
