Thursday, November 14, 2013
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Autoantibodies against BPI fold
containing family B member 1 (BPIFB1)
to diagnose autoimmune interstitial lung disease (ILD)
Patient and mouse studies
suggest autoantibodies against BPIFB1 may help diagnose autoimmune ILD. In a
large cohort of patients with autoimmune polyglandular syndrome type 1
(APS1), an autoantibody to BPIFB1 was detected in 9.6% of patients with APS1
and 100% of patients with both APS1 and ILD. In additional subjects with
idiopathic ILD, the antibody was detected in 12% of patients, whereas the
antibody was not detected in age-matched controls. In Bpifb1 knockout
mice, immunization with recombinant BPIFB1 triggered an immune response, and
transfer of these immune cells into immunodeficient mice led to the
development of a lung disease similar to ILD. Next steps could include using Bpifb1-/-
mouse models to understand the mechanisms underlying autoimmune ILD and to
Published online Nov. 14, 2013
Patent and licensing status
Shum, A.K. et al. Sci.
Transl. Med.; published online Oct. 9, 2013;
Contact: Mark S. Anderson, University of California, San
Contact: Anthony K. Shum, same affiliation as above
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