IMMUNOLOGY2026™ Conference Recordings For Attendees-Autoimmune Dysfunction in FTD-ALS Spectrum Disease

Video Transcription

Video Summary

The speaker presented unpublished work on autoimmune dysfunction and neuroinflammation in frontotemporal dementia-ALS spectrum disease, focusing on progranulin (GRN) and C9orf72 mutations. They noted that FTD often begins before age 65 and differs from Alzheimer’s by affecting behavior, language, and social cognition. Human studies suggest carriers of GRN or C9orf72 mutations may have more autoimmune diseases before neurodegeneration. In mouse models, combined loss of GRN and C9orf72 caused shorter survival, severe peripheral inflammation, glomerulonephritis, vasculitis, splenomegaly, lymphadenopathy, and later motor problems. The double knockout also produced strong brain astrogliosis and microgliosis, blood-brain barrier disruption, and immune-cell uptake of serum proteins. Single-cell studies showed expansion of activated B cells, and antibody profiling identified multiple autoantigens, including ROBO3. Immunizing mice with ROBO3 induced astrocyte activation, while TLR7 inhibition reduced abnormal B-cell activation, anti-ROBO3 antibodies, and astrogliosis. The study supports autoimmune mechanisms as potential biomarkers and therapeutic targets in FTD.

Meta Tag

Date April 18, 2026 12:30 PM - 2:30 PM

Room 104C

Session Autoimmunity, Neurodegeneration and Aging

Speaker Eric Huang

Track Basic Autoimmunity (BA)

Year 2026

Keywords

frontotemporal dementia

autoimmune dysfunction

neuroinflammation

progranulin GRN

C9orf72 mutations

ROBO3 autoantibodies

April 18, 2026 12:30 PM - 2:30 PM

104C

Autoimmunity, Neurodegeneration and Aging

Eric Huang

Basic Autoimmunity (BA)

2026