Uniprot ID: Q00653	Protein name : Nuclear factor NF-kappa-B p100 subunit	Gene : NFKB2 LYT10
Protein Families :	Squence Length : 900

Sequence	>Q00653 901 MESCYNPGLDGIIEYDDFKLNSSIVEPKEPAPETADGPYLVIVEQPKQRGFRFRYGCEGPSHGGLPGASSEKGRKTYPTVKICNYEGPAKIEVDLVTHSDPPRAHAHSLVGKQCSELGICAVSVGPKDMTAQFNNLGVLHVTKKNMMGTMIQKLQRQRLRSRPQGLTEAEQRELEQEAKELKKVMDLSIVRLRFSAFLRASDGSFSLPLKPVISQPIHDSKSPGASNLKISRMDKTAGSVRGGDEVYLLCDKVQKDDIEVRFYEDDENGWQAFGDFSPTDVHKQYAIVFRTPPYHKMKIERPVTVFLQLKRKRGGDVSDSKQFTYYPLVEDKEEVQRKRRKALPTFSQPFGGGSHMGGGSGGAAGGYGGAGGGGSLGFFPSSLAYSPYQSGAGPMGCYPGGGGGAQMAATVPSRDSGEEAAEPSAPSRTPQCEPQAPEMLQRAREYNARLFGLAQRSARALLDYGVTADARALLAGQRHLLTAQDENGDTPLHLAIIHGQTSVIEQIVYVIHHAQDLGVVNLTNHLHQTPLHLAVITGQTSVVSFLLRVGADPALLDRHGDSAMHLALRAGAGAPELLRALLQSGAPAVPQLLHMPDFEGLYPVHLAVRARSPECLDLLVDSGAEVEATERQGGRTALHLATEMEELGLVTHLVTKLRANVNARTFAGNTPLHLAAGLGYPTLTRLLLKAGADIHAENEEPLCPLPSPPTSDSDSDSEGPEKDTRSSFRGHTPLDLTCSTKVKTLLLNAAQNTMEPPLTPPSPAGPGLSLGDTALQNLEQLLDGPEAQGSWAELAERLGLRSLVDTYRQTTSPSGSLLRSYELAGGDLAGLLEALSDMGLEEGVRLLRGPETRDKLPSTAEVKEDSAYGSQSVEQEAEKLGPPPEPPGGLCHGHPQPQVH
Repeat regions	REPEAT 487 519 ANK 1 REPEAT 526 555 ANK 2 REPEAT 559 591 ANK 3 REPEAT 599 628 ANK 4 REPEAT 633 663 ANK 5 REPEAT 667 696 ANK 6 REPEAT 729 758 ANK 7
Sequence region of repeats	487- 519 DTPLHLAIIHGQTSVIEQIVYVIHHAQDLGVVN 526- 555 QTPLHLAVITGQTSVVSFLLRVGADPALLD 559- 591 DSAMHLALRAGAGAPELLRALLQSGAPAVPQLL 599- 628 LYPVHLAVRARSPECLDLLVDSGAEVEATE 633- 663 RTALHLATEMEELGLVTHLVTKLRANVNART 667- 696 NTPLHLAAGLGYPTLTRLLLKAGADIHAEN 729- 758 HTPLDLTCSTKVKTLLLNAAQNTMEPPLTP
Domains	DOMAIN 38 343 RHD. DOMAIN 764 851 Death
Function	"NF-kappa-B is a pleiotropic transcription factor present in almost all cell types and is the endpoint of a series of signal transduction events that are initiated by a vast array of stimuli related to many biological processes such as inflammation, immunity, differentiation, cell growth, tumorigenesis and apoptosis. NF-kappa-B is a homo- or heterodimeric complex formed by the Rel-like domain-containing proteins RELA/p65, RELB, NFKB1/p105, NFKB1/p50, REL and NFKB2/p52. The dimers bind at kappa-B sites in the DNA of their target genes and the individual dimers have distinct preferences for different kappa-B sites that they can bind with distinguishable affinity and specificity. Different dimer combinations act as transcriptional activators or repressors, respectively. NF-kappa-B is controlled by various mechanisms of post-translational modification and subcellular compartmentalization as well as by interactions with other cofactors or corepressors. NF-kappa-B complexes are held in the cytoplasm in an inactive state complexed with members of the NF-kappa-B inhibitor (I-kappa-B) family. In a conventional activation pathway, I-kappa-B is phosphorylated by I-kappa-B kinases (IKKs) in response to different activators, subsequently degraded thus liberating the active NF-kappa-B complex which translocates to the nucleus. In a non-canonical activation pathway, the MAP3K14-activated CHUK/IKKA homodimer phosphorylates NFKB2/p100 associated with RelB, inducing its proteolytic processing to NFKB2/p52 and the formation of NF-kappa-B RelB-p52 complexes. The NF-kappa-B heterodimeric RelB-p52 complex is a transcriptional activator. The NF-kappa-B p52-p52 homodimer is a transcriptional repressor. NFKB2 appears to have dual functions such as cytoplasmic retention of attached NF-kappa-B proteins by p100 and generation of p52 by a cotranslational processing. The proteasome-mediated process ensures the production of both p52 and p100 and preserves their independent function. p52 binds to the kappa-B consensus sequence 5'-GGRNNYYCC-3', located in the enhancer region of genes involved in immune response and acute phase reactions. p52 and p100 are respectively the minor and major form; the processing of p100 being relatively poor. Isoform p49 is a subunit of the NF-kappa-B protein complex, which stimulates the HIV enhancer in synergy with p65. In concert with RELB, regulates the circadian clock by repressing the transcriptional activator activity of the CLOCK-ARNTL/BMAL1 heterodimer"
Motifs	MOTIF 337 341 Nuclear localization signal
Mutation	247 249 YLL->AAA: Two-fold reduction in heterodimerization with RelA 399 399 P->A: No change in cleavage rate or products 404 404 G->A: No change in cleavage rate or products 405 405 A->P: No change in cleavage rate or products 406 406 Q->N: No change in cleavage rate or products 713 713 S->G: Loss of phosphorylation when associated with A-715 and A-717 715 715 S->A: Loss of phosphorylation when associated with G-713 and A-717 717 717 S->A: Loss of phosphorylation when associated with G-713 and A-715 866 866 S->A: Decrease in MAP3K14-induced phosphorylation no inducible processing occurs when associated with A-869 870 870 S->A: Decrease in MAP3K14-induced phosphorylation no inducible processing occurs when associated with A-865
Disease	"DISEASE: Note=A chromosomal aberration involving NFKB2 is found in a case of B-cell non Hodgkin lymphoma (B-NHL) Translocation t(10;14)(q24;q32) with IGHA1 The resulting oncogene is also called Lyt-10C alpha variant ; DISEASE: Note=A chromosomal aberration involving NFKB2 is found in a cutaneous T-cell leukemia (C-TCL) cell line This rearrangement produces the p80HT gene which codes for a truncated 80 kDa protein (p80HT) ; DISEASE: Note=In B-cell leukemia (B-CLL) cell line, LB40 and EB308, can be found after heterogeneous chromosomal aberrations, such as internal deletions ; DISEASE: Immunodeficiency, common variable, 10 (CVID10) [MIM:615577]: A primary immunodeficiency characterized by childhood-onset of recurrent infections, hypogammaglobulinemia, and decreased numbers of memory and marginal zone B-cells Some patients may develop autoimmune features and have circulating autoantibodies An unusual feature is central adrenal insufficiency Note=The disease is caused by mutations affecting the gene represented in this entry"

Q00653 Proteins with ANK Repeats