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Symbol NFKB2 contributors: mct - updated : 11-12-2014
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
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)
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
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrinepancreas   highly
    Nervousbrain   moderately
    Reproductivemale systemprostate  moderately
    Respiratorylung   highly
    Urinarykidney   moderately
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    cell lineage
    cell lines
    at STAGE
  • 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
    interspecies homolog to murine Nfkb2
    FAMILY NF-kappa B/REL family
    CATEGORY DNA associated , transcription factor , protooncogene
    SUBCELLULAR LOCALIZATION     intracellular
  • trapped in the cytoplasm when inactivated,translocated to the nucleus after activation
  • shuttles between the cytoplasm and nucleus
  • NFKB family members NFKB2 and RELB translocate to the nucleus in response to CO2
  • 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
  • critical role for the combined activity of the RELB and NFKB2 subunits in B cell homeostasis that cannot be compensated for by the canonical NFKB pathway under physiological conditions
  • CELLULAR PROCESS nucleotide, transcription
  • 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
    small molecule
  • 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
    inhibited by FBXW7-mediated destruction of NFKB2 is a regulatory component restricting the response to NFKB2 pathway stimulation
    MYC and harnesses MYC-driven lymphomagenesis
    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
    Variant & Polymorphism
    Candidate gene
    Therapy target
    TRAF2/MAP3K14/NFKB2 pathway could be a valuable target for therapy of this cancer