Ask about this productRelated genes to: BCL10 antibody
- 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 antibody
Related articles to: BCL10 antibody
- Cholangiocarcinoma (CCA) is a highly aggressive malignancy with poor prognosis and limited therapeutic options. While Isolinderalactone (ILL) has shown anticancer potential, its effects and underlying mechanisms in CCA remain unclear. This study investigated the anti-CCA activity of ILL and elucidated its molecular mechanisms. ILL significantly inhibited CCA cell viability, proliferation, migration, and invasion, while also inducing G0/G1 cell cycle arrest and promoting apoptosis. Transcriptomic and biochemical analyses revealed that ILL suppressed the NF-κB signaling pathway by inhibiting IκB phosphorylation and p65 activation. Mechanistically, ILL was found to directly bind to BCL10, a key adaptor protein. This interaction disrupted the formation of the CARMA1-BCL10-MALT1 (CBM) signalosome and consequently reduced BCL10 ubiquitination. Functional experiments confirmed this mechanism: BCL10 knockdown mimicked ILL's inhibitory effects, while a BCL10 mutation abolished them. Furthermore, activation of NF-κB with Diprovocim partially reversed ILL's suppressive effects. In a xenograft nude mouse model, ILL effectively reduced tumor growth without significant toxicity and suppressed NF-κB activation in tumor tissues. These findings demonstrate that Isolinderalactone suppresses CCA progression by modulating the CBM signalosome and BCL10 ubiquitination to regulate the NF-κB pathway, supporting its potential as a promising therapeutic candidate for CCA. - Source: PubMed
Publication date: 2026/05/15
Chen WangyangXu DongchaoLiu QiangWang XuehuiXu ChenshanZhu YuanlingLin WenjunZhang HongchenZhang XiaofengYang JianfengShen Hongzhang - Germline variants lead to a spectrum of disease depending on the effect of signalling augmentation or impairment. Autosomal recessive (AR) CARD11 loss-of-function (LOF) is rare with only 12 cases reported to date. - Source: PubMed
Publication date: 2026/05/06
Anderson HamishVerryt CeciliaKeating PaulaSaunders ThomasDalton SamuelHock Barry DGoddard LipingDrake KylieWerno AnjaO'Donnell JohnStinson Jeffrey RSnow Andrew LHsiao Kuang-Chih - Acinar cell carcinoma (ACC) is a lineage-specific carcinoma that occurs nearly exclusively in the pancreas. We report, to our knowledge, the first series of gallbladder carcinoma demonstrating extensive acinar differentiation in the absence of a pancreatic primary. - Source: PubMed
Publication date: 2026/03/29
Yilmaz OsmanAktaş Berk KaanTezcan NurayDeshpande VikramAdsay VolkanVyas Monika - Dysregulation of the CARD11-BCL10-MALT1 (CBM) complex is associated with a group of inborn errors of immunity termed “CBM-opathies,” which encompass a spectrum of clinical manifestations including combined immunodeficiency, autoimmune inflammation, atopic disorders, and lymphoproliferation. In this study, we identified novel compound heterozygous variants in the CBM complex in a patient with a family history of immune dysfunction. The patient inherited the variants CARD11 p.K215N and MALT1 p.K543R/p.M732T from asymptomatic carrier parents. Phenotypically, the patient exhibited a developmental arrest of B lymphocytes at the transitional/naïve B cell stage, accompanied by activation of virus-response pathways. Impaired development of T follicular helper cells was linked to defective germinal center formation and agammaglobulinemia. Furthermore, the patient showed expansion of T peripheral helper cells and a deficiency in regulatory T cells, both associated with autoimmunity and colitis. In vitro studies confirmed an imbalance in Tph/Tfh cell differentiation. Single-cell RNA sequencing further revealed a deficiency in B cell development and an enriched population of pro-inflammatory CD3CD4CD8CD247 T cells, functionally enriched in the MAPK signaling pathway. Mechanistically, the MALT1 K543R and M732T variants attenuated MALT1’s enzymatic activity and compromised its protein stability, while the CARD11 variant disrupted CARD11-mediated promotion of BCL10 filament formation. We demonstrated that these three variants act synergistically to impair NF-κB activation. Specifically, CARD11 cooperates with the co-pathogenic MALT1 and the modifier variant MALT1 to destabilize the functional integrity of the CBM complex, thereby driving the patient’s phenotype. In summary, our study provides new insights into the pathogenesis of autoimmune inflammatory disorders within the spectrum of CBM-opathies and reveals a potential role for the CBM complex in regulating the balance between T peripheral helper and T follicular helper cells. - Source: PubMed
Publication date: 2026/03/26
Li RuiSun XiaochenBao WeiYu HaolanDan YuQingWu ChunmeiMa YanYin HanlinLin WanyiLu LiangjingFu QiongYang Chenghua - Pancreatic acinar cell carcinoma (ACC) is a rare pancreatic malignancy with molecular and clinical features distinct from pancreatic ductal adenocarcinoma (PDAC). We report a 58-year-old man with a 15-cm ACC of the pancreatic body and tail treated by distal pancreatectomy and splenectomy. Complete resection was achieved despite tumor size and surface fissuring suggestive of impending rupture. Operative time was 282 minutes with blood loss of 280 mL. Histopathology showed acinar architecture with B-cell lymphoma 10 (Bcl-10) positivity and nuclear beta-catenin accumulation, consistent with Wnt pathway activation. To clarify the biological differences between ACC and PDAC, we compared their molecular and clinicopathological features. Our analysis demonstrates that ACC's unique molecular profile and growth characteristics enabled successful surgical resection in this giant tumor. This case illustrates how systematic comparison of ACC with PDAC provides insights into the distinct biological behaviors of these pancreatic malignancies. We review current therapeutic advances in PDAC, including targeted therapies and immunotherapy approaches, which may inform future treatment strategies for ACC. - Source: PubMed
Publication date: 2026/02/14
Akiyama RioKawashita YujoOchiai MikuTateishi MasakiUeda TakashiNakamura MasayukiUmeda KoyaHarada SeikoAbe SoseiHaraguchi MasashiYamaguchi JunzoWashida YasuoHachitanda Yoichi