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FLASH GENE
Symbol NFKB2 contributors: mct - updated : 11-12-2013
HGNC name nuclear factor of kappa light polypeptide gene enhancer in B-cells 2 (p49/p100)
HGNC id 7795
Corresponding disease
CVID10 common variable immunodeficiency 10
Location 10q24.32      Physical location : 104.154.228 - 104.162.280
Synonym name lymphocyte translocation chromosome 10
Synonym symbol(s) LYT10, NFKBR, KBF2, EVI140, H2TF1, p100, p52
DNA
TYPE functioning gene
STRUCTURE 8.42 kb     23 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked   status confirmed
Physical map
POLL 10q23 polymerase (DNA directed), lambda DKFZP566F084 SHFM3 10q24 split hand/foot malformation (ectrodactyly) type 3 FGF8 10q24 fibroblast growth factor 8 (androgen-induced) NPM3 10q24.31 nucleophosmin/nucleoplasmin, 3 MGEA5 10q24.1-q24.3 meningioma expressed antigen 5 (hyaluronidase) KCNIP2 10q24 Kv channel interacting protein 2 FLJ13114 10q24.32 hypothetical protein FLJ13114 HPS6 10q24.32 hypothetical protein FLJ13114 LDB1 10q24-q25 LIM domain binding 1 PRC 10q24.32 PGC-1 related co-activator NOLC1 10q22-q25 nucleolar and coiled-body phosphoprotein 1 ELOVL3 10q24.32 elongation of very long chain fatty acids (FEN1/Elo2, SUR4/Elo3, yeast)-like 3 PITX3 10q25 paired-like homeodomain transcription factor 3 GBF1 10q24 golgi-specific brefeldin A resistance factor 1 NFKB2 10q24 nuclear factor of kappa light polypeptide gene enhancer in B-cells 2 (p49/p100) PSD 10q24 pleckstrin and Sec7 domain protein MGC11279 10q24.32 hypothetical protein MGC11279 MGC2491 FLJ14280 10q24.32 hypothetical protein FLJ14280 FLJ22529 10q24.32-q24.33 hypothetical protein FLJ22529 ACTR1A 10q24 ARP1 actin-related protein 1 homolog A, centractin alpha (yeast) SUFU 10q24.32 suppressor of fused homolog (Drosophila) TRIM8 10q24.3 tripartite motif-containing 8 ARL3 10q24.1 ADP-ribosylation factor-like 3 SFXN2 10q24.32 sideroflexin 2 C10orf26 10q24.33 chromosome 10 open reading frame 26 CYP17A1 10q24.3 cytochrome P450, family 17, subfamily A, polypeptide 1 LOC390000 10 similar to Tebp-pending-prov protein C10orf32 10q24.33 chromosome 10 open reading frame 32 CYT19 10q24.33 Cyt19 protein CNNM2 10q24.32 cyclin M2 NT5C2 10q24.32 5'-nucleotidase, cytosolic II LOC90470 10q24.33 similar to Ribulose-phosphate 3-epimerase (Ribulose-5-phosphate-3-epimerase) (HUSSY-17)
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
- - - - 775 in several human tumor cell lines. 2003 12927781
  • p100HB
  • resulting from a point-mutation that generates a premature stop-codon, and thus the protein lacks the last 125 amino acids
  • may be associated with deregulated NF-kappa B/Rel functions
  • 23 - 3123 96.7 900 - 2003 12927781
  • p100
  • inhibiting Rel dimers
  • 22 - 3138 96.6 899 - 2003 12927781
    23 - 3052 96.7 900 - 2003 12927781
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrinepancreas   highly
    Nervousbrain   moderately
    Reproductivemale systemprostate  moderately
    Respiratorylung   highly
    Urinarykidney   moderately
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Epithelialsecretoryglandularendocrine 
    Epithelialsecretoryglandularexocrine 
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a rel homology domain
  • a putative nuclear localization signal (NLS)
  • a glycine rich sequence (poly G)
  • seven ankyrin repeats encoding domain, including a decameric consensus sequence of NFKBS
  • IPT/TIG domain
  • conjugated GlycoP , sumoylated
    mono polymer heteromer , dimer
    isoforms Precursor protein,encoding the p100 precursor and the amino-terminal active p52(p49)subunit
    HOMOLOGY
    interspecies homolog to murine Nfkb2
    Homologene
    FAMILY NF-kappa B/REL family
    CATEGORY DNA associated , transcription factor , protooncogene
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus,nucleoplasm
    text
  • trapped in the cytoplasm when inactivated,translocated to the nucleus after activation
  • shuttles between the cytoplasm and nucleus
  • basic FUNCTION
  • transcription factor of kappa light chain gene enhancer in B cells
  • key role for NF-kappaB2 in the regulation of RELA activation and suggest overlap in the function of NF-kappaB members in canonical and noncanonical pathway signaling
  • regulator of development, differentiation apoptosis
  • NFKB1 and NFKB2 proteins serve both as NF-kappaB precursors and inhibitors of NF-kappaB dimers
  • has a role in the early commitment of CD34+ hematopoietic stem and progenitor cells (HSPCs) towards the myeloid lineage
  • functions as the primary inhibitor of the noncanonical NFKB pathway by sequestering NFKB heterodimers in the cytoplasm
  • principal protein involved in the noncanonical NFKB pathway, evolutionarily conserved, and functioning in peripheral lymphoid organ development, B cell development, and antibody production
  • CELLULAR PROCESS nucleotide, transcription
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
  • RELB/NFKB2 signaling positively and intrinsically regulates hematopoietic stem/progenitor cell (HSPC) self-renewal and maintains stromal/osteoblastic niches
  • a component
  • NFKB complex with REL, RELA or RELB
  • TRAF2/MAP3K14/NFKB2 pathway regulates pancreatic ductal adenocarcinoma (PDAC) cell tumorigenicity and could be a valuable target for therapy of this cancer
  • NFKB2:RELB heterodimer is more adaptable to complement sequence and structural variations in kappaB sites when compared with other NF-kappaB dimers
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • SUMO1 modification of NF-kappaB2 is essential for stimuli-induced NFKB2 phosphorylation and processing
  • represses Sp1-mediated transcription at the CD99 promoter
  • interactor of the F-box protein FBXW7 (FBXW7-dependent degradation of NFKB2 functions as a prosurvival mechanism through control of NFKB activity)
  • cell & other
    REGULATION
    inhibited by FBXW7-mediated destruction of NFKB2 is a regulatory component restricting the response to NFKB2 pathway stimulation
    MYC and harnesses MYC-driven lymphomagenesis
    ASSOCIATED DISORDERS
    corresponding disease(s) CVID10
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral fusion      
    with IGHA in t(10;14)(q24;q32) in B cell non Hodgkin lymphoma
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    cancerendocrinepancreas
    TRAF2/MAP3K14/NFKB2 pathway could be a valuable target for therapy of this cancer
    ANIMAL & CELL MODELS