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FLASH GENE
Symbol RPN1 contributors: mct - updated : 13-03-2019
HGNC name ribophorin I
HGNC id 10381
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
  • expression of ASB11 can increase RPN1 protein turnover (24337577)
  • 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