BCL10
- Known as:
- BCL10
- Catalog number:
- 002484A
- Product Quantity:
- 250ul
- Category:
- -
- Supplier:
- ABM
- Gene target:
- BCL10
Related genes to: BCL10
- Gene:
- BCL10 NIH gene
- Name:
- BCL10 immune signaling adaptor
- Previous symbol:
- -
- Synonyms:
- CARMEN, CIPER, mE10, c-E10, CLAP
- Chromosome:
- 1p22.3
- Locus Type:
- gene with protein product
- Date approved:
- 1999-01-08
- Date modifiied:
- 2019-04-23
Related products to: BCL10
Related articles to: BCL10
- Human genetic disorders affecting intracellular signaling pathways provide an unparalleled opportunity to understand how immune responses are regulated in vivo. Among these conditions, MALT1 deficiency has emerged as a particularly informative model because it reveals how subtle quantitative changes in antigen receptor signaling can translate into profound clinical consequences. As a central component of the CARD11-BCL10-MALT1 (CBM) signalosome, MALT1 integrates receptor-derived signals and determines whether downstream nuclear factor kappa B (NF-kB) activation reaches thresholds required for effective immune responses. Rather than representing a single, uniform loss-of-function condition, MALT1 deficiency is genetically and functionally heterogeneous: different pathogenic variants affect MALT1 protein expression, paracaspase/protease activity, or its scaffolding role within the CBM complex to varying degrees, and these differences determine whether the predominant phenotype reflects impaired immune activation, immune dysregulation, or a combination of both. This functional diversity helps explain why patients with MALT1 deficiency may present with recurrent infections, inflammatory features, regulatory T-cell defects, and progressive impairment of B-cell immunity, either alone or in combination. In this review, we discuss how insights from human disease, experimental models, and therapeutic studies converge to position MALT1 as a critical regulator of immune homeostasis and explore broader implications for translational immunology. Due to the rarity of MALT1 deficiency, we carefully compiled and contextualized the few reported cases of hematopoietic stem cell transplantation (HSCT), highlighting emerging translational patterns. - Source: PubMed
Kose HulyaYalcin KorayKazanci Elif Guler - Copy number alterations (CNAs) drive cancer by amplifying oncogenes and deleting tumor suppressor genes. Although CNA patterns are well-studied in common cancers, they remain poorly characterized in rare tumors. - Source: PubMed
Publication date: 2026/08/14
Pavlov Vladislav SFedorova Maria SElnukaev Turpal-Ali S MKalinin Dmitry VPudova Elena AKatunina Irina VGuvatova Zulfiya GKobelyatskaya Anastasia AKaprin Andrey DKudryavtseva Anna VSnezhkina Anastasiya V - Gastric mucosa-associated lymphoid tissue (MALT) lymphoma, also known clinically as gastric MALT lymphoma (GML) or MALToma, is an indolent B-cell neoplasm strongly associated with chronic Helicobacter pylori () infection. While early-stage disease is based on persistent antigenic stimulation and chronic inflammation, the metabolic and molecular transitions that drive monoclonal B-cell autonomy remain poorly understood. Importantly, maintain this long-term colonization by defusing the host's innate immunity; specifically, its lipid A portion features unique elongated acyl chains, composed of 16-18 carbon atoms, that fail to bind to and activate host TLR4/MD2 receptors, resulting in exceptionally weak endotoxic potency. Persistent colonization relies on key oncoproteins, particularly cytotoxin-associated gene A (CagA) and vacuolar cytotoxin A (VacA), which orchestrate early inflammatory infiltration (neutrophils, Th1, Th2 and Th17 cells) before shifting the microenvironment toward a suppressive regulatory T cell (Treg) phenotype. In this study, we propose a new critical step in the oncogenesis of gastric metastasis: chronic mitochondrial and immunometabolic adaptation within the gastric microenvironment. We claim that act not only as a trigger for infection but also as a chronic driver of mitochondrial adaptation to oxidative stress and hypoxia, which subsequently results in defective mitophagy. CagA- and VacA-mediated mitochondrial damage induces reactive oxygen species (ROS) and functional hypoxia, stabilizing HIF-1α to force a glycolytic metabolic shift, while incomplete mitophagy rescues metabolically altered, apoptosis-resistant clones to drive monoclonal B-cell expansion. Within this ecological-microenvironmental framework, the predominantly cytoplasmic sequestration of BCL10 and the NF-κB subunit p65 observed in GML is reinterpreted not as evidence of signaling inactivity, but as a dynamically regulated adaptive state. This configuration is orchestrated by mitochondrial stress responses that enable adaptation to the chronic microenvironmental pressures imposed by , acting in concert with the metabolic programs governed by MYC, NRF2, and BCL2. Overall, this review outlines the multi-step pathogenesis of -mediated GML, highlighting how mitochondrial dysfunction and metabolic remodeling drive the transition from chronic infection to malignant transformation. - Source: PubMed
Publication date: 2026/08/05
Gargiulo Isacco CiroPham Van HungPham Huong ThienNguyen Kieu Cao DiemTran Toai CongLe Thach HuyJirillo FelicitaJirillo EmilioSantacroce Luigi - Pancreatic amphicrine-like carcinoma (ALC) is an exceptionally rare neoplasm characterized by simultaneous exocrine and endocrine differentiation within the same tumour cells. These tumours represent a diagnostic challenge because they must be distinguished from mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs), which consist of morphologically distinct tumour components. We report a case of pancreatic ALC with acinar differentiation harboring a KANK4::RAF1 fusion identified by comprehensive genomic profiling. Histologically, the tumour demonstrated acinar differentiation with expression of trypsin and BCL10 together with neuroendocrine differentiation characterized by synaptophysin and INSM1 expression within the same neoplastic population. Molecular analysis revealed a RAF1 rearrangement, a potentially actionable alteration previously described in a subset of pancreatic acinar carcinomas. The patient showed rapid disease progression despite systemic chemotherapy. Treatment with the MEK inhibitor trametinib was initiated based on the presence of a RAF1 fusion but was discontinued after 1 month because of toxicity, preventing assessment of therapeutic efficacy. This case expands the molecular spectrum of pancreatic ALC with acinar differentiation and highlights the importance of comprehensive molecular profiling in rare pancreatic neoplasms to identify potentially actionable genomic alterations. - Source: PubMed
Publication date: 2026/08/11
Kammerer SabrinaBode PeterSchreiber HeideOrtega Sanchez Guacimara - Epstein-Barr virus (EBV) is a human herpesvirus that causes a variety of hematological malignancies, in particular B-, T- and NK-cell lymphomas. Latency proteins of the virus contribute to lymphomagenesis by activating the transcription factor NF-κB, however, the molecular mechanism underlying EBV-driven NF-κB activation remains incompletely understood. Here we show that EBV latent membrane proteins LMP1 and LMP2A or LMP2B synergize to activate the protease MALT1, a key driver of NF-κB-mediated lymphocyte proliferation. EBV-positive B-cell lines co-expressing LMP1 and LMP2 proteins exhibited constitutive MALT1 activity, and treatment with a MALT1 inhibitor or silencing of latent membrane protein-1 (LMP1) or LMP2 impaired MALT1-dependent substrate cleavage. LMP1 physically interacted with the MALT1-binding partner BCL10, while LMP2A and LMP2B interacted with MALT1. LMP1 also colocalized with LMP2 in dotted structures, suggesting that LMP1 and LMP2 synergize via the coordinated formation of LMP-BCL10-MALT1 (LBM) complexes. Finally, MALT1 protease inhibition led to reduced expression of a set of NF-κB-driven genes, and reduced tumor growth in an EBV-positive lymphoma xenograft model. These findings establish a key role for the protease MALT1 in LMP1/2-mediated, NF-κB-driven cellular transformation and provide a rationale for inhibiting MALT1 to treat EBV-positive B-cell lymphomas that co-express LMP1 and LMP2. - Source: PubMed
Publication date: 2026/08/13
Juilland MélanieRomy LaurenceRashid Harun-OrLopes Sara EscudeiroPodavini SilviaVallois DavidLuo ManhuiGonzalez MontserratPandeva KaterinaFrête FrédéricDécaillet Chantalde Leval LaurenceRothenberger SylviaThome Margot