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FLASH GENE
Symbol BCL10 contributors: mct - updated : 14-10-2016
HGNC name B-cell CLL/lymphoma 10
HGNC id 989
Corresponding disease
IMD37 Immunodeficiency 37
MALTL mucosa-associated lymphoid tissue (MALT) lymphomas
Location 1p22.3      Physical location : 85.731.460 - 85.742.587
Synonym name
  • CARD- containing molecule enhancing NF-kappa-B
  • CARD-like apoptotic protein
  • mammalian CARD-containing adapter molecule E10
  • caspase-recruiting domain-containing protein
  • CARD-containing proapoptotic protein
  • CED-3/ICH-1 prodomain homologous E10-like regulator
  • cellular homolog of vCARMEN
    • Synonym symbol(s) CLAP, CARMEN, ME10, CIPER, C-E10, EVI57
    DNA
    TYPE functioning gene
    STRUCTURE 12.13 kb     4 Exon(s)
    Genomic sequence alignment details
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    4 - 3118 - 233 - 2008 18806265
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularvessel    
    Lymphoid/Immunelymph node   highly
    Reproductivefemale systembreastmammary gland highly
    Urinarybladder   highly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a N terminal caspase recruitement domain (CARD), and a 13-AAs region downstream of the CARD domain is responsible for interacting with the immunoglobulin-like domains of MALT1
  • a C terminal region rich in serine and threonine residues
    • mono polymer homomer , heteromer , dimer
    HOMOLOGY
    interspecies homolog to murine Bcl10
    Homologene
    FAMILY
    CATEGORY regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,lysosome
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus
    text perinuclear structures when bound to CARD11
    basic FUNCTION
  • positive regulator of lymphocyte proliferation that specifically connects antigen receptor signaling in B and T cells to NFKB activation
  • positive regulator of cell apoptosis
  • essential mediator of LPA (lysophosphatidic)-induced NF-kappaB activation
  • potential transcriptional activator that interacts with general transcription factor GTF2B
  • key signaling component mediating NF-B activation induced by G protein-coupled receptors in nonlymphoid cells
  • forms a complex with MALT1 to activate the IKK complex through ubiquitination of the IKK/NEMO subunit of the IKK complex in antigen receptor signaling pathways
  • CARD10 and BCL10 contributed to several characteristics of EGFR-associated malignancy, including proliferation, survival, migration, and invasion
  • CARD11/BCL10/MALT1 (CBM) signalosome mediates antigen receptor-induced NFKB1 signaling to regulate multiple lymphocyte functions
  • CARD11, BCL10, and MALT1, that have complex 5'UTRs and encode proteins with short half-lives, are involved in BCR signaling
    • CELLULAR PROCESS cell life, cell death/apoptosis
    PHYSIOLOGICAL PROCESS
    text promoting apoptosis and suppressing malignant transformation in vitro
    PATHWAY
    metabolism
    signaling signal transduction
    pathway of NF-kappa B activation, CARD10-BCL10-MALT1 signaling pathway mediates NF-B activation in response to antigen receptor stimulation in lymphocytes
    a component
  • forming a ternary complex with CARD10, MALT1
  • CARD11-BCL10-MALT1 (CBM) complex connects T-cell receptor (TCR) signalling to the canonical IkappaB kinase (IKK)/NF (nuclear factor)-kappaB pathway
  • CARD9-BCL10-MALT1 pathway activated by SYK
  • CARD10-BCL10-MALT1 signalosome promotes angiotensin II-dependent vascular inflammation and atherogenesis
    • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • CARD11
  • death domain of MALT1 and the CARD of BCL10 also contribute to BCL10-MALT1 interactions
  • IL2-independent STAT5B target gene
  • interacting with CALM1 (CALM1 regulates T cell responses to antigens by binding to BCL10, thereby modulating its interaction with CARD11 and subsequent activation of NF-kappaB)
  • interacting with CAMK2G (phosphorylates the CARD domain of BCL10, which regulates the interactions within the important CARD11, BCL10, MALT1 signalling complex)
  • CARD11 serves as a scaffold for BCL10, MALT1 and other effector proteins and regulates various signaling pathways related to the immune response
  • interaction mode between CARD11 CARD and BCL10 CARD based on a structure-based modeling study
  • ATM-dependent phosphorylation of BCL10 promotes its interaction with and presentation of UBE2N to RNF8, and RNF8-mediated ubiquitination of BCL10 enhances binding of BCL10 and UBE2N to RNF168
  • novel function for CRADD in endothelial cells as an inducible suppressor of BCL10, a key mediator of responses to proinflammatory agonists
  • AIP augments CARMA1-BCL10-MALT1 complex formation to facilitate NFKB1 signaling upon T cell activation
  • CARD14 recruits BCL10 and MALT1 to form the CARD-BCL10-MALT1 complex, which modulates NFKB1 and MAPK signalling pathway
    • cell & other
  • viral E10
    • REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MALTL , IMD37
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral   translocation    
    t (1;14)(p22;q32) as uncommon but recurrent events in MALT lymphoma
    tumoral   deletion    
    involving 1, 10, 17 chromosome in Sezary syndrome and chromosome instability
    tumoral fusion      
    an IGH-BCL2-fusion with t(1;14)(p22;q32), in MALT lymphoma
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
    Bcl 10-/- mice (Ruland 01)