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FLASH GENE
Symbol RPN1 contributors: mct - updated : 13-03-2019
HGNC name ribophorin I
HGNC id 10381
Location 3q21.3      Physical location : 128.338.812 - 128.369.719
Synonym name
  • dolichyl-diphosphooligosaccharide-protein glycosyltransferase 67 kDa subunit
  • hetero-oligomeric oligosaccharyltransferase 1
  • Synonym symbol(s) RBPH1, OST1, DKFZp686B16177
    EC.number 2.4.1.119/2.4.99.18
    DNA
    TYPE functioning gene
    STRUCTURE 30.91 kb     10 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Physical map
    DKFZp564A176 3q21.3 hypothetical protein DKFZp564A176 LOC285311 3q21.3 hypothetical gene supported by AK097460 TPRA40 3q21.2 seven transmembrane domain orphan receptor MCM2 3q21 MCM2 minichromosome maintenance deficient 2, mitotin (S. cerevisiae) PODLX2 15 endoglycan ABTB1 3q21 ankyrin repeat and BTB (POZ) domain containing 1 MGLL 3q21.3 monoglyceride lipase LOC166348 3q21.3 similar to RIKEN cDNA 4833415F11 SEC61A1 3q21.3 similar to RIKEN cDNA 4833415F11 RUVBL1 3q21 RuvB-like 1 (E. coli) SELB 3q21.3 elongation factor for selenoprotein translation LOC389146 3 LOC389146 DNAJB8 3q21.3 DnaJ (Hsp40) homolog, subfamily B, member 8 GATA2 3q21 GATA binding protein 2 LOC391572 3 similar to Transmembrane protein Tmp21 precursor (21 kDa Transmembrane trafficking protein) (p24delta) (S31III125) (S31I125) (Tmp-21-I) GR6 3q21 similar to Transmembrane protein Tmp21 precursor (21 kDa Transmembrane trafficking protein) (p24delta) (S31III125) (S31I125) (Tmp-21-I) FLJ40473 3q21.3 hypothetical protein FLJ40473 RPN1 3q21.3-q25.2 ribophorin I RAB7 3q21 RAB7, member RAS oncogene family LOC391573 3 similar to mKIAA1860 protein LOC391574 3 similar to MAP/microtubule affinity-regulating kinase 4; MAP/microtubule affinity-regulating kinase 4L; MARK4 serine/threonine protein kinase LOC389147 3 similar to WD repeat domain 10 isoform 3 LOC391575 3 similar to Fat-specific protein FSP27 homolog ACAD9 3q21.3 acyl-Coenzyme A dehydrogenase family, member 9 KIAA1257 3q21.3 KIAA1257 protein FLJ12057 3q21.3 hypothetical protein FLJ12057 GP9 3q21 glycoprotein IX (platelet) LOC339122 KIAA1160 3q21.3 KIAA1160 protein ZNF9 3p13.3-q24 zinc finger protein 9 (a cellular retroviral nucleic acid binding protein) COPG 3q21.3 coatomer protein complex, subunit gamma DC12 3q21.3 DC12 protein H1FX 3q21.3 H1 histone family, member X LOC90288 3q21.3 hypothetical protein LOC90288 MBD4 3q21-q22 methyl-CpG binding domain protein 4 WDR10 3q21 WD repeat domain 10 RHO 3q21.3-q24 rhodopsin (opsin 2, rod pigment) (retinitis pigmentosa 4, autosomal dominant) H1FOO 3q21.3 H1 histone family, member O, oocyte-specific PLXND1 3q21.3 plexin D1 KIAA0779 3q21.3 plexin D1 TRH 3q13.3-q21 thyrotropin-releasing hormone LOC391576 3 hypothetical gene supported by AK122996 LOC285407 3q21.3 similar to mannosyltransferase LOC339944 3q21.3 similar to hypothetical protein FLJ10661 LOC389148 3 LOC389148
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    10 - 2372 - 607 - 2008 18607003
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart    
    Digestiveliver   highly
    Endocrinepancreas   highly
    Nervousbrain   highly
    Reproductivemale systemprostate  highly
    Respiratorylung    
    Urinarykidney   highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / Hematopoieticbone marrow   
    Muscularstriatumskeletal  
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • N-glycosylation site within its luminal domain
  • conjugated GlycoP
    HOMOLOGY
    interspecies ortholog to murine Rpn1
    homolog to Drosophila rho-5
    homolog to C.elegans T22D1.4
    Homologene
    FAMILY
    CATEGORY chaperone/stress , enzyme , RNA associated
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    intracellular,cytoplasm,cytosolic
    text type I membrane protein
    basic FUNCTION
  • transfering a high mannose oligosaccharide from a lipid-linked oligosaccharide donor onto asparagine accaptor sites within an Asn-X-Ser/Thr consensus motif in newly synthesized proteins
  • facilitate the N-glycosylation of certain precursors during their biogenesis at the endoplasmic reticulum
  • can regulate the delivery of precursor proteins to the oligosaccharyltransferase complex by capturing substrates and presenting them to the catalytic core
  • acts as a substrate-specific facilitator of N-glycosylation
  • involved in asparagine-linked glycosylation of polypeptides in the lumen of the endoplasmic reticulum
  • distinct and complementary roles for the RPN1 isoforms STT3A, STT3B allowing sequential scanning of polypeptides for acceptor sites to insure the maximal efficiency of N-glycosylation
  • having a novel chaperone activity that is a consequence of N-glycosylation-dependent direct interaction with OPRM1
  • RPN1 functions as a chaperone inside the cell
  • may play a central role in the selective association between MLEC and misfolded glycoproteins
  • may function as a chaperone that recognizes misfolded proteins inside cells
  • CELLULAR PROCESS protein, post translation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • subunit of oligosaccharyltransferase
  • part of an N-oligosaccharyl transferase complex that links high mannose oligosaccharides to asparagine residues
  • oligomeric complex of three nonidentical subunits of 67 kDa (ribophrin I),63/64 kDa (ribophorin II) and 48 kDa
  • STT3A, STT3B, are RPN1 soform
  • MLEC forms a stable complex with RPN1
  • INTERACTION
    DNA
    RNA binding
    small molecule
    protein
  • could directly interact with OPRM1
  • UBLCP1 is recruited to the proteasome through direct interaction between its UBL domain and RPN1
  • association of malectin with ribophorin I is required to capture misfolded glycoproteins and direct them to the degradation pathway
  • subcellular localization of malectin is accurately regulated by the expression level of RPN1
  • cell & other binding to ribosome
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral fusion      
    with JAK2 in t(3;9)(q21;p24) in a patient with chronic idiopathic myelofibrosis (CIMF), a chronic myeloproliferative disorder
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS