(7) RNF204 (Kr.19p13.3), ZNRF4 , Nixin; PA-TM-RING RNF-alaperhe . ERAD mekanismilla Calnexiinin säätö

RNF204 ZNRF4, nixin, ovat  suositeltuja nimiä. Hiiriortologesita tulee nimet

SPERIZIN, SPZN, Ssrzf1 PA-TM-RING RNF alaryhmä

Etsittäessä ERAD-linkkiytyneen proteiinidegradaation ja ER funktion uusia säätelijätekijöitä, löydettiin kirjo endoplasmiseen retikulumiin lokalisoituneita E3 ubikitiiniligaaseja ja 24 oli ankkuroituneena endoplasmiseen retikulumiin (ER). Niiden joukosta löytyi tämä nixin/ZNRF4 Se näytti olevan keskeinen calnexiinin (CNX) vaihtuvuuden säätelijä. ERAD = Endoplasmiseen retikulumiin assosioitunut degradaatio. Wt nixiinin ektooppinen ilmenemä indusoi vahvan alassäädön endoplasmiseen retikulumiin lokalisoituneessa kaitsijaproteiinissa (chaperone)  kalnexiini (CNX) nimeltään. Nixiini teki siihen suoran interaktion glykosylaatiosta riippuvalla tavalla, indusoi sen ubikitinaation ja p97:stä riippuvan hajoittamisen - mikä viittaa ERAD mekanismiin. http://www.abcam.com/index.html?pageconfig=resource&rid=324

https://www.ncbi.nlm.nih.gov/gene/148066

GENE Also known as

spzn; RNF204; Ssrzf1; SPERIZIN

Orthologs mouse all

Related articles in PubMed

1. A systematic search for endoplasmic reticulum (ER) membrane-associated RING finger proteins identifies Nixin/ZNRF4 as a regulator of calnexin stability and ER homeostasis.
2. Neutzner A, et al. J Biol Chem, 2011 Mar 11. PMID 21205830, Free PMC Article
   To identify novel regulators of endoplasmic reticulum (ER)-linked (ERAD) protein degradation and ER function, we determined the entire inventory of membrane-spanning RING finger E3 ubiquitin ligases localized to the ER. We identified 24 ER membrane-anchored ubiquitin ligases and found Nixin/ZNRF4 to be central for the regulation of calnexin turnover. Ectopic expression of wild type Nixin induced a dramatic down-regulation of the ER-localized chaperone calnexin that was prevented by inactivation of the Nixin RING domain. Importantly, Nixin physically interacts with calnexin in a glycosylation-independent manner, induces calnexin ubiquitination, and p97-dependent degradation, indicating an ER-associated degradation-like mechanism of calnexin turnover.
3. Sperizin is a murine RING zinc-finger protein specifically expressed in Haploid germ cells. Fujii T, et al. Genomics, 1999 Apr 1. PMID 10191088
4. A comprehensive framework of E2-RING E3 interactions of the human ubiquitin-proteasome system. van Wijk SJ, et al. Mol Syst Biol, 2009. PMID 19690564, Free PMC Article
5. The DNA sequence and biology of human chromosome 19. Grimwood J, et al. Nature, 2004 Apr 1. PMID 15057824
6. Architecture of the human interactome defines protein communities and disease networks. Huttlin EL, et al. Nature, 2017 May 25. PMID 28514442, Free PMC Article
   

See all (8) citations in PubMed
See citations in PubMed for homologs of this gene provided by HomoloGene

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

1. Nixin/ZNRF4 to be central for the regulation of calnexin turnover.
2. Characterization and study of mouse homolog.
   

Related articles in PubMed

1. A systematic search for endoplasmic reticulum (ER) membrane-associated RING finger proteins identifies Nixin/ZNRF4 as a regulator of calnexin stability and ER homeostasis. Neutzner A, et al. J Biol Chem, 2011 Mar 11. PMID 21205830, Free PMC Article
2. Sperizin is a murine RING zinc-finger protein specifically expressed in Haploid germ cells. Fujii T, et al. Genomics, 1999 Apr 1. PMID 10191088
3. A comprehensive framework of E2-RING E3 interactions of the human ubiquitin-proteasome system. van Wijk SJ, et al. Mol Syst Biol, 2009. PMID 19690564, Free PMC Article
4. The DNA sequence and biology of human chromosome 19. Grimwood J, et al. Nature, 2004 Apr 1. PMID 15057824
5. Architecture of the human interactome defines protein communities and disease networks. Huttlin EL, et al. Nature, 2017 May 25. PMID 28514442, Free PMC Article
   

See all (8) citations in PubMed
See citations in PubMed for homologs of this gene provided by HomoloGene

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

1. Nixin/ZNRF4 to be central for the regulation of calnexin turnover.
2. Characterization and study of mouse homolog.
   

Preferred Names

E3 ubiquitin-protein ligase ZNRF4

Names

RING finger protein 204

RING-type E3 ubiquitin transferase ZNRF4

nixin

zinc/RING finger protein 4

Conserved Domains (2) summary

cd02123
Location:86 → 244

PA_C_RZF_like; PA_C-RZF_ like: Protease-associated (PA) domain C_RZF-like. This group includes various PA domain-containing proteins similar to C-RZF (chicken embryo RING zinc finger) protein. These proteins contain a C3H2C3 RING finger. C-RZF is expressed in embryo ...

pfam13639
Location:307 → 352

zf-RING_2; Ring finger domain

E3 ubiquitin-protein ligase ZNRF4 precursor [Homo sapiens]

NCBI Reference Sequence: NP_859061.3
Identical Proteins FASTA Graphics

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LOCUS       NP_859061                429 aa            linear   PRI 29-APR-2018
DEFINITION  E3 ubiquitin-protein ligase ZNRF4 precursor [Homo sapiens].
ACCESSION   NP_859061
VERSION     NP_859061.3
DBSOURCE    REFSEQ: accession NM_181710.3
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 429)
  AUTHORS   Neutzner A, Neutzner M, Benischke AS, Ryu SW, Frank S, Youle RJ and
            Karbowski M.
  TITLE     A systematic search for endoplasmic reticulum (ER)
            membrane-associated RING finger proteins identifies Nixin/ZNRF4 as
            a regulator of calnexin stability and ER homeostasis
  JOURNAL   J. Biol. Chem. 286 (10), 8633-8643 (2011)
   PUBMED   21205830
  REMARK    GeneRIF: Nixin/ZNRF4 to be central for the regulation of calnexin
            turnover.
REFERENCE   2  (residues 1 to 429)
  AUTHORS   Grimwood J, Gordon LA, Olsen A, Terry A, Schmutz J, Lamerdin J,
            Hellsten U, Goodstein D, Couronne O, Tran-Gyamfi M, Aerts A,
            Altherr M, Ashworth L, Bajorek E, Black S, Branscomb E, Caenepeel
            S, Carrano A, Caoile C, Chan YM, Christensen M, Cleland CA,
            Copeland A, Dalin E, Dehal P, Denys M, Detter JC, Escobar J,
            Flowers D, Fotopulos D, Garcia C, Georgescu AM, Glavina T, Gomez M,
            Gonzales E, Groza M, Hammon N, Hawkins T, Haydu L, Ho I, Huang W,
            Israni S, Jett J, Kadner K, Kimball H, Kobayashi A, Larionov V,
            Leem SH, Lopez F, Lou Y, Lowry S, Malfatti S, Martinez D, McCready
            P, Medina C, Morgan J, Nelson K, Nolan M, Ovcharenko I, Pitluck S,
            Pollard M, Popkie AP, Predki P, Quan G, Ramirez L, Rash S, Retterer
            J, Rodriguez A, Rogers S, Salamov A, Salazar A, She X, Smith D,
            Slezak T, Solovyev V, Thayer N, Tice H, Tsai M, Ustaszewska A, Vo
            N, Wagner M, Wheeler J, Wu K, Xie G, Yang J, Dubchak I, Furey TS,
            DeJong P, Dickson M, Gordon D, Eichler EE, Pennacchio LA,
            Richardson P, Stubbs L, Rokhsar DS, Myers RM, Rubin EM and Lucas
            SM.
  TITLE     The DNA sequence and biology of human chromosome 19
  JOURNAL   Nature 428 (6982), 529-535 (2004)
   PUBMED   15057824
REFERENCE   3  (residues 1 to 429)
  AUTHORS   Fujii T, Tamura K, Copeland NG, Gilbert DJ, Jenkins NA, Yomogida K,
            Tanaka H, Nishimune Y, Nojima H and Abiko Y.
  TITLE     Sperizin is a murine RING zinc-finger protein specifically
            expressed in Haploid germ cells
  JOURNAL   Genomics 57 (1), 94-101 (1999)
   PUBMED   10191088
  REMARK    GeneRIF: Characterization and study of mouse homolog.
COMMENT     VALIDATED REFSEQ: This record has undergone validation or
            preliminary review. The reference sequence was derived from
            AC005764.1.
            On Jun 30, 2007 this sequence version replaced NP_859061.2.
            
            Sequence Note: The RefSeq transcript and protein were derived from
            genomic sequence to make the sequence consistent with the reference
            genome assembly. The genomic coordinates used for the transcript
            record were based on alignments.
FEATURES             Location/Qualifiers
     source          1..429
                     /organism="Homo sapiens"
                     /db_xref="taxon:9606"
                     /chromosome="19"
                     /map="19p13.3"
     Protein         1..429
                     /product="E3 ubiquitin-protein ligase ZNRF4 precursor"
                     /EC_number="2.3.2.27"
                     /note="zinc/RING finger protein 4; RING finger protein
                     204; nixin; E3 ubiquitin-protein ligase ZNRF4; RING-type
                     E3 ubiquitin transferase ZNRF4"
                     /calculated_mol_wt=44039
     sig_peptide     1..27
                     /experiment="experimental evidence, no additional details
                     recorded"
                     /note="{ECO:0000255}; propagated from UniProtKB/Swiss-Prot
                     (Q8WWF5.3)"
                     /calculated_mol_wt=2938
     mat_peptide     28..429
                     /product="E3 ubiquitin-protein ligase ZNRF4"
                     /experiment="experimental evidence, no additional details
                     recorded"
                     /note="propagated from UniProtKB/Swiss-Prot (Q8WWF5.3)"
                     /calculated_mol_wt=44039
     Region          86..244
                     /region_name="PA_C_RZF_like"
                     /note="PA_C-RZF_ like: Protease-associated (PA) domain
                     C_RZF-like. This group includes various PA
                     domain-containing proteins similar to C-RZF (chicken
                     embryo RING zinc finger) protein. These proteins contain a
                     C3H2C3 RING finger. C-RZF is expressed in embryo...;
                     cd02123"
                     /db_xref="CDD:239038"
     Site            194..196
                     /site_type="other"
                     /note="PA/protease or protease-like domain interface
                     [polypeptide binding]"
                     /db_xref="CDD:239038"
     Site            251..271
                     /site_type="transmembrane region"
                     /experiment="experimental evidence, no additional details
                     recorded"
                     /note="propagated from UniProtKB/Swiss-Prot (Q8WWF5.3)"
     Region          307..352
                     /region_name="zf-RING_2"
                     /note="Ring finger domain; pfam13639"
                     /db_xref="CDD:290367"
     Site            order(309,312,327,329,332,335,348,351)
                     /site_type="other"
                     /note="cross-brace motif"
                     /db_xref="CDD:238093"
     CDS             1..429
                     /gene="ZNRF4"
                     /gene_synonym="RNF204; SPERIZIN; spzn; Ssrzf1"
                     /coded_by="NM_181710.3:78..1367"
                     /db_xref="CCDS:CCDS42475.1"
                     /db_xref="GeneID:148066"
                     /db_xref="HGNC:HGNC:17726"
                     /db_xref="MIM:612063"
ORIGIN      
        1 mplcrpehlm prasrvpvaa slplshavip tqlpsrpghr ppgrprrcpk asclpppvgp
       61 sstqtakrvt mgwprpgral vavkallvls llqvpaqavv ravlednsss vdfadlpalf
      121 gvplapegir gylmevkpan achpieaprl gnrslgaivl irrydctfdl kvlnaqragf
      181 eaaivhnvhs ddlvsmthvy edlrgqiaip svfvseaasq dlrvilgcnk sahalllpdd
      241 ppchdlgchp vltvswvlgc tlalvvsaff vlnhlwlwaq accshrrpvk tstcqkaqvr
      301 tftwhndlca icldeyeegd qlkilpcsht yhckcidpwf sqaprrscpv ckqsvaated
      361 sfdsttysfr dedpslpghr ppiwaiqvql rsrrlellgr asphchcstt sleaeyttvs
      421 sappeapgq
//