IMMUNOLOGY2026™ Conference Recordings For Attendees-CBM-opathies: New insights on lymphocyte metabolism and effector function

Video Transcription

Video Summary

The speaker discussed CARD11 and the CBM complex, key scaffolds in B and T-cell signaling that activate NF-kappaB, JNK, and mTOR pathways. He reviewed three CARD11-related immune disorders: complete CARD11 deficiency, which causes combined immunodeficiency; gain-of-function mutations causing BENTA disease with B-cell lymphoproliferation; and dominant-negative hypomorphic mutations causing CEDAN/“cadence,” marked mainly by severe atopy, high IgE, eosinophilia, infections, and sometimes autoimmunity. The lab showed that cadence mutations impair JNK signaling, increasing NFATC1 and GATA3, which may drive Th2 skewing. They also found CBM signaling controls nutrient transporters ASCT2 and GLUT1, linking CARD11 defects to reduced glutamine/glucose uptake and weaker mTORC1 activity. Restoring these transporters partly corrected T-cell polarization. The talk ended with potential therapies, including IL-4 blockade, sirolimus, MALT1 inhibitors, and possibly glutamine supplementation.

Meta Tag

Date April 18, 2026 9:05 AM - 9:25 AM

Room 104C

Session Surprising Insights on Human Immunology from the Study of Monogenic Inborn Errors of Immunity, Sponsored by the Clin. Immunol. Soc. (CIS)

Speaker Andrew Snow

Track Human/translational immunology

Year 2026

Keywords

CARD11

CBM complex

NF-kappaB signaling

BENTA disease

CEDAN cadence

mTORC1 metabolism

April 18, 2026 9:05 AM - 9:25 AM

104C

Surprising Insights on Human Immunology from the Study of Monogenic Inborn Errors of Immunity, Sponsored by the Clin. Immunol. Soc. (CIS)

Andrew Snow

Human/translational immunology

2026