Publications by authors named "Franziska Ober"

CARD-BCL10-MALT1 (CBM) signalosomes connect distal signaling of innate and adaptive immune receptors to proximal signaling pathways and immune activation. Four CARD scaffold proteins (CARD9, 10, 11, 14) can form seeds that nucleate the assembly of BCL10-MALT1 filaments in a cell- and stimulus-specific manner. MALT1 (also known as PCASP1) serves a dual function within the assembled CBM complexes.

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Constitutive mucosa-associated lymphoid tissue lymphoma translocation protein 1 (MALT1) activity drives survival of malignant lymphomas addicted to chronic B-cell receptor signaling, oncogenic CARD11, or the API2-MALT1 (also BIRC3::MALT1) fusion oncoprotein. Although MALT1 scaffolding induces NF-κB-dependent survival signaling, MALT1 protease function is thought to augment NF-κB activation by cleaving signaling mediators and transcriptional regulators in B-cell lymphomas. However, the pathological role of MALT1 protease function in lymphomagenesis is not well understood.

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Article Synopsis
  • - MALT1 deficiency is a serious immune disorder leading to recurrent infections, chronic diarrhea, eczema, and growth issues in patients, which has not been extensively studied in large groups before.
  • - A study evaluated nine new patients alongside ten previously reported cases, revealing that most had normal T and NK cells but reduced B cells, with a high rate of severe complications and mortality linked to infections.
  • - The findings suggest that hematopoietic stem cell transplantation (HSCT) may improve survival rates, indicating it should be considered a primary treatment option for affected individuals early in life.
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