ELM
The Eukaryotic Linear Motif resource for
Functional Sites in Proteins

LIG_CSL_BTD_1

Accession:
Functional site class:
Ligand motif binding the CSL BTD
Functional site description:
The DNA binding β-trefoil domain is built out of a capped β-barrel, which has an internal pseudo threefold symmetry. In CSL proteins this domain functions as an interaction site for NotchIC (the intracellular post-proteolytic part of the Notch receptor), co-repressors and some viral regulatory proteins such as EBNA2 and EBNA3. CSL (CBF1/RBP-jκ, Su(H), Lag-1) proteins serve as co-repressors or as co-activators of Notch pathway target gene expression. CSL’s regulatory character changes from a repressing to an activating component when the RAM domain of NotchIC binds to it. This interaction with NotchIC is necessary for the expression of genes that are important in embryonic developmental processes and tissue maintenance.
ELM Description:
The ɸWɸP motif represents the main interaction site between Notch-like proteins and CSL transcription factors. This interaction plays an important role in the Notch pathway. The very conserved W and P in the CSL binding-motif enter into a hydrophobic pocket provided by CSL(Wilson,2006).The other two positions are less conserved but both also have hydrophobic preferences. Since CSL may function as either a gene expression repressor or activator, depending on it’s conformation, it represents a key step in gene expression where several co-regulatory proteins operate. RITA and KyoT2 are such co-regulators and interact with CSL via the same binding motif as Notch, ɸWɸP (Wacker,2011; Collins,2014).
Some viruses have developed mechanisms to manipulate Notch-signalling by expressing proteins that carry this binding motif and thus permanently stimulate Notch-like expression regulation (Calderwood,2011; Heinzelmann,2010).
In all non-viral proteins, which carry the motif, a conserved occurrence of a G or an A at least 3 positions in front of the SLiM can be noticed. In Notch proteins, a basic region of circa five amino acids length precedes the hydrophobic motif, separated by a gap of about five residues. The basic residues also bind to CSL.
The viral proteins, which tend to manipulate Notch-controlled gene-expression, do not show these extra residues, having just the core WxP-based motif. Often the viral proteins have multiple WxP motifs, some of which do not match the ELM pattern, having a non-hydrophobic residue in the less conserved positions. It is likely that the viral motifs can bind multiple CSL molecules.
Pattern: [AFILMPTVW]W[FHILMPSTVW]P
Pattern Probability: 0.0001278
Present in taxons: Metazoa Viruses
Interaction Domain:
BTD (PF09270) Beta-trefoil DNA-binding domain (Stochiometry: 1 : 1)
o See 18 Instances for LIG_CSL_BTD_1
In metazoa, the highly conserved Notch pathway plays an important role in embryonic development, cell fate determination, and maintenance processes. When the single-pass transmembrane Notch receptor binds to its ligand, Delta, a membrane-bound protein of neighbouring cells, three proteolytic cleavages take place and lead to the release of NotchIC, the intracellular domain and effector of Notch (18363556).NotchIC then translocates into the nucleus and regulates transcription of target genes by recruiting cofactors and assembling with them into the regulation-complex. The complex consists of intracellular Notch, the co-activating protein, Mastermind, and the transcription factor CSL (CBF1/RBPjκ; Su(H); Lag-1 20972443). In the complex, the β-trefoil domain (BTD) of CSL interacts with the RBPJ-associated molecule (RAM) intrinsically disordered domain (IDD) of the Notch protein.
This interaction is mainly managed by a short hydrophobic xWxP amino acid sequence in the Notch like proteins(Notch1, P46531; Notch2, Q04721; Notch3, Q9UM47; Notch4, Q99466; Lin-12, P14585; GLP1, P13508), which is highly conserved across species. In the complex, the W and P of the motif are directed into a hydrophobic pocket of the CSL BTD domain (Wilson,2006, 2FO1).
CSL also interacts with the corepressor KyoT2, A2AEX7 (Collins,2014) and RITA, RBP-J interacting and tubulin associated, P0CJ62 (Wacker,2011) via this motif. RITA exports CSL from the nucleus. Ataxin1, P5253 (Tong,2011) may also have the motif, though it has not been shown to be functional.
EBNA2, Q3KSV2 (Calderwood,2011); EBNA3, Q3KST2 (Calderwood,2011); EBNA6, P03204 (8627785) and K10, F5H9D8 (Heinzelmann,2010) are viral proteins that bind to CSL and thus take effect on gene-regulation of Notch-target genes. The viral proteins often have repeated xWxP motifs, suggesting that they bind multiple copies of CSL.
o 8 selected references:


o 13 GO-Terms:
Biological Process:
Organ Regeneration (also annotated in class: )
Transcription, Dna-Dependent (also annotated in these classes: DOC_PP1_SILK_1 LIG_14-3-3_CanoR_1 LIG_14-3-3_CterR_2 ELM:old_LIG_14-3-3_2 )
Development (also annotated in these classes: LIG_Sin3_1 LIG_Sin3_2 LIG_Sin3_3 MOD_ASX_betaOH_EGF )
Cellular Response To Corticosteroid Stimulus (also annotated in class: )
Regulation Of Transcription, Dna-Dependent (also annotated in these classes: DEG_COP1_1 DEG_ODPH_VHL_1 DOC_MAPK_DCC_7 DOC_MAPK_GRA24_9 DOC_MAPK_HePTP_8 DOC_MAPK_JIP1_4 DOC_MAPK_MEF2A_6 DOC_MAPK_NFAT4_5 LIG_ANK_PxLPxL_1 LIG_NBox_RRM_1 LIG_RBL1_LxSxE_2 )
Cell Differentiation (also annotated in these classes: CLV_C14_Caspase3-7 LIG_SH2_CRK LIG_SH2_SFK_CTail_3 LIG_WRPW_1 LIG_WRPW_2 MOD_ASX_betaOH_EGF )
Notch Signaling Pathway (also annotated in class: )
Regulation Of Cell Migration (also annotated in these classes: LIG_PTB_Apo_2 LIG_PTB_Phospho_1 )
Cellular Compartment:
Chromatin (also annotated in these classes: LIG_LEDGF_IBM_1 LIG_RB_LxCxE_1 )
Transcription Factor Complex (also annotated in these classes: DOC_MAPK_DCC_7 DOC_MAPK_MEF2A_6 LIG_HCF-1_HBM_1 LIG_Menin_MBM1_1 LIG_RB_pABgroove_1 )
Nucleus (also annotated in these classes: CLV_C14_Caspase3-7 CLV_Separin_Fungi CLV_Separin_Metazoa CLV_TASPASE1 DEG_APCC_DBOX_1 DEG_APCC_KENBOX_2 DEG_APCC_TPR_1 DEG_Cend_DCAF12_1 DEG_Cend_FEM1AC_1 DEG_Cend_FEM1B_2 DEG_Cend_KLHDC2_1 DEG_Cend_TRIM7_1 DEG_COP1 DEG_COP1_1 DEG_CRL4_CDT2_1 DEG_CRL4_CDT2_2 DEG_Kelch_Keap1_1 DEG_Kelch_Keap1_2 DEG_MDM2_SWIB_1 DEG_ODPH_VHL_1 DEG_SCF_COI1_1 DEG_SCF_FBW7_1 DEG_SCF_FBW7_2 DEG_SCF_FBXO31_1 DEG_SCF_SKP2-CKS1_1 DEG_SCF_TIR1_1 DEG_SCF_TRCP1_1 DEG_SIAH_1 DEG_SPOP_SBC_1 DOC_ANK_TNKS_1 DOC_CDC14_PxL_1 DOC_CKS1_1 DOC_CYCLIN_D_Helix_1 DOC_CYCLIN_RevRxL_6 DOC_CYCLIN_RxL_1 DOC_CYCLIN_yClb1_LxF_4 DOC_CYCLIN_yClb3_PxF_3 DOC_CYCLIN_yClb5_NLxxxL_5 DOC_CYCLIN_yCln2_LP_2 DOC_MAPK_DCC_7 DOC_MAPK_FxFP_2 DOC_MAPK_gen_1 DOC_MAPK_GRA24_9 DOC_MAPK_HePTP_8 DOC_MAPK_JIP1_4 DOC_MAPK_MEF2A_6 DOC_MAPK_NFAT4_5 DOC_MAPK_RevD_3 DOC_PIKK_1 DOC_PP1_MyPhoNE_1 DOC_PP1_RVXF_1 DOC_PP1_SILK_1 DOC_PP2A_B56_1 DOC_PP2A_KARD_1 DOC_PP2B_LxvP_1 DOC_PP2B_PxIxIT_1 DOC_PP4_FxxP_1 DOC_PP4_MxPP_1 DOC_USP7_MATH_1 DOC_USP7_MATH_2 DOC_USP7_UBL2_3 DOC_WW_Pin1_4 LIG_14-3-3_CanoR_1 LIG_14-3-3_ChREBP_3 LIG_14-3-3_CterR_2 LIG_ANK_PxLPxL_1 LIG_APCC_ABBA_1 LIG_APCC_Cbox_1 LIG_APCC_Cbox_2 LIG_ARL_BART_1 LIG_ARS2_EDGEI_1 LIG_BRCT_BRCA1_1 LIG_BRCT_BRCA1_2 LIG_BRCT_MDC1_1 LIG_CaM_1-14-15-16_REV_1 LIG_CaMK_CASK_1 LIG_CORNRBOX LIG_CtBP_PxDLS_1 LIG_CtBP_RRT_2 LIG_DCNL_PONY_1 LIG_EF_ALG2_ABM_1 LIG_EF_ALG2_ABM_2 LIG_EH1_1 LIG_FHA_1 LIG_FHA_2 LIG_GLEBS_BUB3_1 LIG_HCF-1_HBM_1 LIG_HOMEOBOX LIG_HP1_1 LIG_IRF7_LxLS_2 LIG_IRFs_LxIS_1 LIG_KEPE_1 LIG_KEPE_2 LIG_KEPE_3 LIG_LEDGF_IBM_1 LIG_LSD1_SNAG_1 LIG_MAD2 LIG_Menin_MBM1_1 LIG_MLH1_MIPbox_1 LIG_MSH2_SHIPbox_1 LIG_MTR4_AIM_1 LIG_Mtr4_Air2_1 LIG_Mtr4_Trf4_1 LIG_Mtr4_Trf4_2 LIG_MYND_1 LIG_MYND_2 LIG_MYND_3 LIG_NBox_RRM_1 LIG_NRBOX LIG_Nrd1CID_NIM_1 LIG_PALB2_WD40_1 LIG_PCNA_APIM_2 LIG_PCNA_PIPBox_1 LIG_PCNA_TLS_4 LIG_PCNA_yPIPBox_3 LIG_PTAP_UEV_1 LIG_RBL1_LxSxE_2 LIG_RB_LxCxE_1 LIG_RB_pABgroove_1 LIG_REV1ctd_RIR_1 LIG_RPA_C_Plants LIG_RPA_C_Vert LIG_RRM_PRI_1 LIG_Rrp6Rrp47_Mtr4_1 LIG_Sin3_1 LIG_Sin3_2 LIG_Sin3_3 LIG_SUFU_1 LIG_SUMO_SIM_anti_2 LIG_SUMO_SIM_par_1 LIG_TPR LIG_Trf4_IWRxY_1 LIG_TRFH_1 LIG_UBA3_1 LIG_ULM_U2AF65_1 LIG_VCP_SHPBox_1 LIG_VCP_VBM_3 LIG_VCP_VIM_2 LIG_WD40_WDR5_VDV_1 LIG_WD40_WDR5_VDV_2 LIG_WD40_WDR5_WIN_1 LIG_WD40_WDR5_WIN_2 LIG_WD40_WDR5_WIN_3 LIG_WRPW_1 LIG_WRPW_2 LIG_WW_2 MOD_AAK1BIKe_LxxQxTG_1 MOD_CDC14_SPxK_1 MOD_CDK_SPK_2 MOD_CDK_SPxK_1 MOD_CDK_SPxxK_3 MOD_CK1_1 MOD_CK2_1 MOD_DYRK1A_RPxSP_1 MOD_GSK3_1 MOD_NEK2_1 MOD_NEK2_2 MOD_PIKK_1 MOD_PKA_1 MOD_PKA_2 MOD_PKB_1 MOD_PLK MOD_Plk_1 MOD_Plk_2-3 MOD_Plk_4 MOD_PRMT_GGRGG_1 MOD_ProDKin_1 MOD_SUMO_for_1 MOD_SUMO_rev_2 ELM:old_LIG_14-3-3_1 ELM:old_LIG_14-3-3_2 ELM:old_LIG_14-3-3_3 TRG_NES_CRM1_1 TRG_NESrev_CRM1_2 TRG_NLS_Bipartite_1 TRG_NLS_MonoCore_2 TRG_NLS_MonoExtC_3 TRG_NLS_MonoExtN_4 )
Molecular Function:
Transcription Factor (also annotated in these classes: LIG_HCF-1_HBM_1 LIG_NBox_RRM_1 LIG_RB_LxCxE_1 )
Protein Domain Specific Binding (also annotated in these classes: DEG_APCC_DBOX_1 DEG_APCC_KENBOX_2 DEG_Cend_DCAF12_1 DEG_Cend_FEM1AC_1 DEG_Cend_FEM1B_2 DEG_Cend_KLHDC2_1 DEG_Cend_TRIM7_1 DEG_Kelch_actinfilin_1 DEG_Kelch_Keap1_1 DEG_Kelch_Keap1_2 DEG_Kelch_KLHL3_1 DOC_MIT_MIM_1 LIG_14-3-3_CanoR_1 LIG_14-3-3_CterR_2 LIG_AP_GAE_1 LIG_ARS2_EDGEI_1 LIG_BRCT_BRCA1_1 LIG_BRCT_BRCA1_2 LIG_BRCT_MDC1_1 LIG_CAP-Gly_1 LIG_CSK_EPIYA_1 LIG_deltaCOP1_diTrp_1 LIG_EH_1 LIG_EVH1_1 LIG_EVH1_2 LIG_EVH1_3 LIG_FHA_1 LIG_FHA_2 LIG_G3BP_FGDF_1 LIG_MSH2_SHIPbox_1 LIG_MTR4_AIM_1 LIG_PAM2_1 LIG_PAM2_2 LIG_PDZ_Class_1 LIG_PDZ_Class_2 LIG_PDZ_Class_3 LIG_PDZ_Wminus1_1 LIG_PTB_Apo_2 LIG_PTB_Phospho_1 LIG_RRM_PRI_1 LIG_SH3_1 LIG_SH3_2 LIG_SH3_3 LIG_SH3_4 LIG_SH3_PxRPPK_7 LIG_SH3_PxxDY_5 LIG_SH3_PxxPPRxxK_8 LIG_SH3_PxxxRxxKP_6 LIG_Trf4_IWRxY_1 LIG_TRFH_1 LIG_ULM_U2AF65_1 LIG_WH1 ELM:old_LIG_14-3-3_1 ELM:old_LIG_14-3-3_2 ELM:old_LIG_14-3-3_3 )


o 18 Instances for LIG_CSL_BTD_1
(click table headers for sorting; Notes column: =Number of Switches, =Number of Interactions)
Acc., Gene-, NameStartEndSubsequenceLogic#Ev.OrganismNotes
Q6AW86 ZNF324B
Z324B_HUMAN
59 62 IQLERGEEPWVPSGKDMTLA TP 2 Homo sapiens (Human)
P03204 EBNA6
EBNA6_EBVB9
96 99 LDTEDNVPPWLPPHDITPYT TP 2 Human herpesvirus 4 (strain B95-8) (Epstein-Barr virus (strain B95-8))
1
Q3KST2 EBNA3
EBNA3_EBVG
772 775 LHQPPMEGPWVPEQWMFQGA TP 6 Human herpesvirus 4 (Epstein-Barr virus)
1
Q3KST2 EBNA3
EBNA3_EBVG
583 586 ARERWRPAPWTPNPPRSPSQ TP 6 Human herpesvirus 4 (Epstein-Barr virus)
1
Q3KSV2 EBNA2
EBNA2_EBVG
289 292 HHLPSGPPWWPPICDPPQPS TP 6 Human herpesvirus 4 (Epstein-Barr virus)
1
P0CJ62 rita1
RITA1_XENLA
142 145 DTVKIRPLLWSPSPRLVQQS TP 6 Xenopus laevis (African clawed frog)
P0CJ62 rita1
RITA1_XENLA
70 73 APPSQTPLLWSPGEIKENKK TP 6 Xenopus laevis (African clawed frog)
A2AEX7 Fhl1
A2AEX7_MOUSE
190 193 NKGLVKAPVWWPMKDNPGTT TP 3 Mus musculus (House mouse)
P13508 glp-1
GLP1_CAEEL
803 806 KRKMVNATVWMPPMESTNEK TP 1 Caenorhabditis elegans
F5H9D8 vIRF-4
F5H9D8_HHV8
203 206 TEQKAACSVWIPVNEGASTS TP 2 Human herpesvirus 8
P46531 NOTCH1
NOTC1_HUMAN
1767 1770 KRRRQHGQLWFPEGFKVSEA TP 4 Homo sapiens (Human)
1
P54253 ATXN1
ATX1_HUMAN
44 47 DNHRVEGTAWLPGNPGGRGH TP 1 Homo sapiens (Human)
F5H9D8 vIRF-4
F5H9D8_HHV8
487 490 VVPEVRTPLWIPWSSGGAPN TP 2 Human herpesvirus 8
F5H9D8 vIRF-4
F5H9D8_HHV8
306 309 GSRLPSTSPWIPACFPWGDL TP 2 Human herpesvirus 8
P14585 lin-12
LIN12_CAEEL
944 947 KRRMINASVWMPPMENEEKN TP 2 Caenorhabditis elegans
Q99466 NOTCH4
NOTC4_HUMAN
1481 1484 RRRREHGALWLPPGFTRRPR TP 1 Homo sapiens (Human)
Q9UM47 NOTCH3
NOTC3_HUMAN
1674 1677 RRKREHSTLWFPEGFSLHKD TP 1 Homo sapiens (Human)
Q04721 NOTCH2
NOTC2_HUMAN
1709 1712 KRKRKHGSLWLPEGFTLRRD TP 1 Homo sapiens (Human)
Please cite: ELM-the Eukaryotic Linear Motif resource-2024 update. (PMID:37962385)

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