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torsdag 3 maj 2018

TRIM1 (Kr.Xq22.3), MID2, MRX101, RNF60 (C_I, COS, FN3, PRYSPRY)

TRIM1 ( Kr.Xq22.3), MID2, MRX101, RNF60, Todennäköisesti E3 ubiquitin-proteiini ligaasi, (C-I, COS, FN3, PRYSPRY)
TRIM1 geeni sijaitsee X-kromosomissa. Proteiini paikallistuu sytoplasmisiin mikrotubuluksiin. Geenin vaihtoehtoisluennat johtavat kahteen erilaiseen transkriptiin ja vastaaviin isoformeihin. C-terminaalin perusteella luokitellaan alaryhmään C_I Tähän geeniin liittyy proteiinin homodimerisaatio, aktivoituminen ja ligaasisaktiviteetti. Geenin tärkeä paralogi on MID1. Geenillä lienee merktitystä mikrotubulien stabilisoinnissa. Vaihtoehtoisin pleissauksin tulee kaksi transkriptia, jotka koodaavat eri isoformeja.Geeniä ilmentyy kilpirauhasessa, rasvakudoksessa  ja 24 muussa kudoksessa

The protein encoded by this gene is a member of the tripartite motif (TRIM) family. The TRIM motif includes three zinc-binding domains, a RING, a B-box type 1 and a B-box type 2, and a coiled-coil region. The protein localizes to microtubular structures in the cytoplasm. Alternate splicing of this gene results in two transcript variants encoding different isoforms. [provided by RefSeq, Feb 2009] Expression Ubiquitous expression in thyroid (RPKM 3.8), fat (RPKM 3.4) and 24 other tissues See more

Suomennosta: MID2-geenin nimi tulee sanasta Midline 2. Sen geenin taudit ovat liittyneet X-kromosomaaliseen mentaaliretardaatioon X-linked 101 ja epäspesifiseen X-linkkiytyneeseen intelligenssin heikkouteen.

RAKENNE: Konservoidut domeenit:

Conserved Domains (5) summary
smart00502
Location:239 → 364
BBC; B-Box C-terminal domain
smart00336
Location:195 → 232
BBOX; B-Box-type zinc finger
cd00063
Location:401 → 531
FN3; Fibronectin type 3 domain; One of three types of internal repeats found in the plasma protein fibronectin. Its tenth fibronectin type III repeat contains an RGD cell recognition sequence in a flexible loop between 2 strands. Approximately 2% of all ...
cd13739
Location:536 → 705
SPRY_PRY_TRIM1; PRY/SPRY domain of tripartite motif-binding protein 1 (TRIM1) or MID2
cd16754
Location:30 → 82
RING-HC_MID2; RING finger, HC subclass, found in midline-2 (MID2) and similar proteins

TRIM1 isoformi 1 (735 aminohappoa)

https://www.ncbi.nlm.nih.gov/protein/NP_036348.2
LOCUS       NP_036348                735 aa            linear   PRI 08-APR-2018
DEFINITION  probable E3 ubiquitin-protein ligase MID2 isoform 1 [Homo sapiens].
ACCESSION   NP_036348
VERSION     NP_036348.2
DBSOURCE    REFSEQ: accession NM_012216.3
KEYWORDS    RefSeq.
SOURCE      Homo sapiens (human)
  ORGANISM  Homo sapiens
            Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
            Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini;
            Catarrhini; Hominidae; Homo.
REFERENCE   1  (residues 1 to 735)
  AUTHORS   Wang L, Wu J, Yuan J, Zhu X, Wu H and Li M.
  TITLE     Midline2 is overexpressed and a prognostic indicator in human
            breast cancer and promotes breast cancer cell proliferation in
            vitro and in vivo
  JOURNAL   Front Med 10 (1), 41-51 (2016)
   PUBMED   26791755
  REMARK    GeneRIF: overexpressed in advanced breast cancer and high
            overexpression is prognostic factor for poor overall survival
REFERENCE   2  (residues 1 to 735)
  AUTHORS   Gholkar AA, Senese S, Lo YC, Vides E, Contreras E, Hodara E, Capri
            J, Whitelegge JP and Torres JZ.
  TITLE     The X-Linked-Intellectual-Disability-Associated Ubiquitin Ligase
            Mid2 Interacts with Astrin and Regulates Astrin Levels to Promote
            Cell Division
  JOURNAL   Cell Rep 14 (2), 180-188 (2016)
   PUBMED   26748699
  REMARK    GeneRIF: Mid2 regulates cell division through the ubiquitination of
            astrin on K409, which is critical for its degradation and proper
            cytokinesis.
REFERENCE   3  (residues 1 to 735)
  AUTHORS   Kimura,T., Jain,A., Choi,S.W., Mandell,M.A., Schroder,K.,
            Johansen,T. and Deretic,V.
  TITLE     TRIM-mediated precision autophagy targets cytoplasmic regulators of
            innate immunity
  JOURNAL   J. Cell Biol. 210 (6), 973-989 (2015)
   PUBMED   26347139
REFERENCE   4  (residues 1 to 735)
  AUTHORS   Geetha TS, Michealraj KA, Kabra M, Kaur G, Juyal RC and Thelma BK.
  TITLE     Targeted deep resequencing identifies MID2 mutation for X-linked
            intellectual disability with varied disease severity in a large
            kindred from India
  JOURNAL   Hum. Mutat. 35 (1), 41-44 (2014)
   PUBMED   24115387
  REMARK    GeneRIF: A novel missense mutation (c.1040G>A, p.Arg347Gln) was
            reported in MID2, which encodes ubiquitin ligase E3, as the likely
            cause of X-linked mental retardation in a large kindred.
REFERENCE   5  (residues 1 to 735)
  AUTHORS   Uchil PD, Hinz A, Siegel S, Coenen-Stass A, Pertel T, Luban J and
            Mothes W.
  TITLE     TRIM protein-mediated regulation of inflammatory and innate immune
            signaling and its association with antiretroviral activity
  JOURNAL   J. Virol. 87 (1), 257-272 (2013)
   PUBMED   23077300
REFERENCE   6  (residues 1 to 735)
  AUTHORS   Yap MW, Nisole S, Lynch C and Stoye JP.
  TITLE     Trim5alpha protein restricts both HIV-1 and murine leukemia virus
  JOURNAL   Proc. Natl. Acad. Sci. U.S.A. 101 (29), 10786-10791 (2004)
   PUBMED   15249690
REFERENCE   7  (residues 1 to 735)
  AUTHORS   Short KM, Hopwood B, Yi Z and Cox TC.
  TITLE     MID1 and MID2 homo- and heterodimerise to tether the
            rapamycin-sensitive PP2A regulatory subunit, alpha 4, to
            microtubules: implications for the clinical variability of X-linked
            Opitz GBBB syndrome and other developmental disorders
  JOURNAL   BMC Cell Biol. 3, 1 (2002)
   PUBMED   11806752
  REMARK    GeneRIF: MID2 coiled-coil motifs mediate both homo- and
            heterodimerization, a prerequisite for association of the MID-alpha
            4 complex with microtubules.
REFERENCE   8  (residues 1 to 735)
  AUTHORS   Reymond A, Meroni G, Fantozzi A, Merla G, Cairo S, Luzi L,
            Riganelli D, Zanaria E, Messali S, Cainarca S, Guffanti A, Minucci
            S, Pelicci PG and Ballabio A.
  TITLE     The tripartite motif family identifies cell compartments
  JOURNAL   EMBO J. 20 (9), 2140-2151 (2001)
   PUBMED   11331580
REFERENCE   9  (residues 1 to 735)
  AUTHORS   Perry J, Short KM, Romer JT, Swift S, Cox TC and Ashworth A.
  TITLE     FXY2/MID2, a gene related to the X-linked Opitz syndrome gene
            FXY/MID1, maps to Xq22 and encodes a FNIII domain-containing
            protein that associates with microtubules
  JOURNAL   Genomics 62 (3), 385-394 (1999)
   PUBMED   10644436
REFERENCE   10 (residues 1 to 735)
  AUTHORS   Buchner G, Montini E, Andolfi G, Quaderi N, Cainarca S, Messali S,
            Bassi MT, Ballabio A, Meroni G and Franco B.
  TITLE     MID2, a homologue of the Opitz syndrome gene MID1: similarities in
            subcellular localization and differences in expression during
            development
  JOURNAL   Hum. Mol. Genet. 8 (8), 1397-1407 (1999)
   PUBMED   10400986
COMMENT     REVIEWED REFSEQ: This record has been curated by NCBI staff. The
            reference sequence was derived from DN997921.1, Y18880.1 and
            AL034399.6.
            This sequence is a reference standard in the RefSeqGene project.
            On Feb 20, 2009 this sequence version replaced NP_036348.1.
            
            Summary: The protein encoded by this gene is a member of the
            tripartite motif (TRIM) family. The TRIM motif includes three
            zinc-binding domains, a RING, a B-box type 1 and a B-box type 2,
            and a coiled-coil region. The protein localizes to microtubular
            structures in the cytoplasm. Alternate splicing of this gene
            results in two transcript variants encoding different isoforms.
            [provided by RefSeq, Feb 2009].
            
            Transcript Variant: This variant (1) represents the longer
            transcript and encodes the longer isoform (1).
            
            Sequence Note: This RefSeq record was created from transcript and
            genomic sequence data to make the sequence consistent with the
            reference genome assembly. The genomic coordinates used for the
            transcript record were based on transcript alignments.
            
            Publication Note:  This RefSeq record includes a subset of the
            publications that are available for this gene. Please see the Gene
            record to access additional publications.
            
            ##Evidence-Data-START##
            Transcript exon combination :: SRR1660805.85252.1, Y18880.1
                                           [ECO:0000332]
            RNAseq introns              :: single sample supports all introns
                                           SAMEA1965299, SAMEA1968189
                                           [ECO:0000348]
            ##Evidence-Data-END##
FEATURES             Location/Qualifiers
     source          1..735
                     /organism="Homo sapiens"
                     /db_xref="taxon:9606"
                     /chromosome="X"
                     /map="Xq22.3"
     Protein         1..735
                     /product="probable E3 ubiquitin-protein ligase MID2
                     isoform 1"
                     /EC_number="2.3.2.27"
                     /note="tripartite motif protein 1; midin 2; probable E3
                     ubiquitin-protein ligase MID2; midline defect 2; RING
                     finger protein 60; tripartite motif-containing protein 1;
                     RING-type E3 ubiquitin transferase MID2"
                     /calculated_mol_wt=83080
     Region          30..82
                     /region_name="RING-HC_MID2"
                     /note="RING finger, HC subclass, found in midline-2 (MID2)
                     and similar proteins; cd16754"
                     /db_xref="CDD:319668"
     Region          30..79
                     /region_name="RING-HC finger (C3HC4-type)"
                     /note="RING-HC finger (C3HC4-type) [structural motif]"
                     /db_xref="CDD:319668"
     Site            order(30,33,45,47,50,53,76,79)
                     /site_type="other"
                     /note="Zn binding site [ion binding]"
                     /db_xref="CDD:319668"
     Region          195..232
                     /region_name="BBOX"
                     /note="B-Box-type zinc finger; smart00336"
                     /db_xref="CDD:197662"
     Site            order(195,198,218,224)
                     /site_type="other"
                     /note="Zn2+ binding site [ion binding]"
                     /db_xref="CDD:237988"
     Region          239..364
                     /region_name="BBC"
                     /note="B-Box C-terminal domain; smart00502"
                     /db_xref="CDD:128778"
     Region          401..531
                     /region_name="FN3"
                     /note="Fibronectin type 3 domain; One of three types of
                     internal repeats found in the plasma protein fibronectin.
                     Its tenth fibronectin type III repeat contains an RGD cell
                     recognition sequence in a flexible loop between 2 strands.
                     Approximately 2% of all...; cd00063"
                     /db_xref="CDD:238020"
     Region          536..705
                     /region_name="SPRY_PRY_TRIM1"
                     /note="PRY/SPRY domain of tripartite motif-binding protein
                     1 (TRIM1) or MID2; cd13739"
                     /db_xref="CDD:293974"
     CDS             1..735
                     /gene="MID2"
                     /gene_synonym="FXY2; MRX101; RNF60; TRIM1"
                     /coded_by="NM_012216.3:574..2781"
                     /note="isoform 1 is encoded by transcript variant 1"
                     /db_xref="CCDS:CCDS14532.2"
                     /db_xref="GeneID:11043"
                     /db_xref="HGNC:HGNC:7096"
                     /db_xref="MIM:300204"
ORIGIN      
        1 mgespasvvl nasgglfslk metleseltc piclelfedp lllpcahslc fscahrilvs
       61 scssgesiep itafqcptcr yvislnhrgl dglkrnvtlq niidrfqkas vsgpnspses
      121 rrertyrptt amsseriacq fceqdpprda vktcitcevs ycdrclrath pnkkpftshr
      181 lvepvpdthl rgitcldhen ekvnmycvsd dqlicalckl vgrhrdhqva slndrfeklk
      241 qtlemnltnl vkrnselenq makliqicqq vevntamhea klmeecdelv eiiqqrkqmi
      301 avkiketkvm klrklaqqva ncrqclerst vlinqaehil kendqarflq sakniaerva
      361 matassqvli pdinfndafe nfaldfsrek kllegldylt apnppsiree lctashdtit
      421 vhwisddefs issyelqyti ftgqanfisk swcswglwpe irkckeavsc srlagaprgl
      481 ynsvdswmiv pnikqnhytv hglqsgtryi fivkainqag srnseptrlk tnsqpfkldp
      541 kmthkklkis ndglqmekde sslkkshtpe rfsgtgcyga agnifidsgc hywevvmgss
      601 twyaigiayk sapknewigk nasswvfsrc nsnfvvrhnn kemlvdvpph lkrlgvlldy
      661 dnnmlsfydp anslhlhtfd vtfilpvcpt ftiwnkslmi lsglpapdfi dyperqecnc
      721 rpqespyvsg mktch
//

Artikkeleita Related articles in PubMed

 TRIM1(MID2, RNF60)  ja BRCA1(RNF53)

Midline2 (MID2) on ubikitiiniä konjugoiva E2-entsyymi, joka linkkiytyy tuumorin progredioitumiseen ja on uusi havaittu proteiini, joka tekee interaktion BRCA1:n kanssa (BRCA1 on RING finger proteiini 53 RNF53, muita nimiä PPP1R53, IMP, IRIS, PSCP, FANCS,PNCA4) . Tässä tutkimuksessa haluttiin selvittää TRIM1:n osuus rintasyövän varhaisessa alussa. MID2 mRNA oli ylössäätynyt yli 95%:ssa tutkituista näytteistä. MID2-ilmenemä myös lisääntyi taudin kliinisen vaikeusasteen myötä. Analyysien perusteella MID2 on itsenäinen prognostinen faktori. . MID2:n siRNA- hiljennys vähensi merkitsevästi syöpäsolulinjoja in vitro ja koe-eläimen xenokraftituumoria in vivo. MID2 voi toimia prognostisena markkerina sekä interventiokohteena rintasyövässä.
  • Midline2 (MID2) is an ubiquitin-conjugating E2 enzyme linked to tumor progression and a novel interacting partner of breast cancer 1, early-onset (BRCA1). However, the role of MID2 in breast cancer remains unknown. This study investigated the expression, prognostic value, and role of MID2 in breast cancer.
  • The expression of MID2 mRNA and protein was significantly upregulated in breast cancer tissue and established cell lines compared with that in normal breast epithelial cells and paired adjacent non-tumor tissue (P < 0.001). Immunohistochemical analysis demonstrated that MID2 was overexpressed in 272 of 284 (95.8%) paraffinembedded, archived breast cancer tissue. Moreover, MID2 expression increased with advanced clinical stage (P < 0.001). High MID2 expression was significantly associated with advanced clinical stages and T, N, and M staging (all P < 0.05). Univariate and multivariate analyses indicated that high MID2 expression was an independent prognostic factor..Results showed that siRNA silencing of MID2 expression significantly reduced MCF-7 and MDA-MB-231 cell proliferation in vitro and blocked the growth of MDA-MB-231 cell xenograft tumors in vivo (P < 0.05). This study indicated that MID2 may be a novel prognostic marker and interventional target in breast cancer.

  TRIM1 (MID2) ja ASTRIINI  

Mid2 regulates cell division through the ubiquitination of astrin on K409, which is critical for its degradation and proper cytokinesis.

  • MID1 ja MID2 ovat ubikitiiniligaaseja, jotka säätelevät mikrotubulusten dynamiikkaa
    ja joiden mutaatiot liittyvät X-kromosomiin linkkiytyneisiin kehityksellisiin häiriöihin.
    Tutkijat osoittivat tässä työssään, että ASTRIINI- niminen proteiini, joka organisoi mikrotubuluksia, saostui tutkittaessa MID1 ja MID2 -proteiinien kanssa. ASTRIININ sijoittuminen kattaa MID1- ja MID2- lokalisaatiokohdat jakaantumassa olevien solujen välisiltojen mikrotubuluksissa. ASTRIININ ubikitinoi lysiiniin (K409) MID2 ja sytokineesin aikana ASTRIINI tämän takia hajoaa normaalisti. Mutta jos tehdään MID2-depleetio ( poisto) , ASTRIINI ehtii stabiloitua sytokineesin kuluessa ja sytokineesi tulee puutteeliseksi, esiintyy monitumaisia soluja ja solukuolemaa ( siis solu ei pääse normaalisti tekemään kahta symmetristä tytärsolua) . Entä jos ASTRIINI itse on mutatoitunut siten, että tarvitava lysiinikohta K409 onkin muuttunut alaniiniksi, joka ei voi ubikitinoitua) Mutatoitunutta ASTRIINIA alkaa tällöin kertyä soluun jakaantumista yrittävien solujen väliseen mikrotubulussiltaan ja niin sytokineesi edelleen vaikeutuu. Solut jäävät monitumaisiksi ja kuolevat. Tulokset osoittavat, että MID2 säätelee solun jakaantumista ubikitinoimalla ASTRIININ K409 lysiinini , mikä on ratkaisevaa, jotta ASTRIINI hajoaisi ajoissa ja solut vapautuisivat normaaliin sytokinetiikkaan. Nämä tulokset saattavat auttaa selittämään, kuinka MID2-mutaatio johtaa mikrotubulusten hyvän järjestyksen sekasortoon ja siitä johtuen signaloinnin alavirrassa X-kromosomiin linkkiytyneeseen tautipatologiaan, joka koskee lopulta ilmenee intelligenssin heikentymänä.
  • Mid1 and Mid2 are ubiquitin ligases that regulate microtubule dynamics and whose mutation is associated with X-linked developmental disorders. We show that astrin, a microtubule-organizing protein, co-purifies with Mid1 and Mid2, has an overlapping localization with Mid1 and Mid2 at intercellular bridge microtubules, is ubiquitinated by Mid2 on lysine 409, and is degraded during cytokinesis. Mid2 depletion led to astrin stabilization during cytokinesis, cytokinetic defects, multinucleated cells, and cell death. Similarly, expression of a K409A mutant astrin in astrin-depleted cells led to the accumulation of K409A on intercellular bridge microtubules and an increase in cytokinetic defects, multinucleated cells, and cell death. These results indicate that Mid2 regulates cell division through the ubiquitination of astrin on K409, which is critical for its degradation and proper cytokinesis. These results could help explain how mutation of MID2 leads to misregulation of microtubule organization and the downstream disease pathology associated with X-linked intellectual disabilities.

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