the role of the goodpasture antigen binding protein in autoimmunity

The Goodpasture antigen-binding protein (GPBP) is a serine/threonine kinase
that binds and phosphorylates collagen type IV, which has a major role in the
organisation of the glomerular basement membrane and is the target of
autoantibodies mediating glomerulonephritis in Goodpasture syndrome. Several
studies have suggested a link between GPBP and the immune system. GPBP has been
shown to be upregulated in autoimmune conditions, to transport the signalling
lipid ceramide, to be regulated by immune signalling molecules and to interact
with pentraxins, the key activators of the complement system. However, the
exact role of GPBP in regulation of the (auto)immune response still remains
unclear. Therefore, we intend to investigate the exact role of GPBP in the
activation and modulation of the immune system.

Primary Objective: characterization of GPBP expression on different subsets of
human leukocytes
Secondary Objective: functional characterization of GPBP on these cells

10 ml of venous blood will be obtained from patients with an autoimmune disease
or another inflammatory disease. Leukocytes will be isolated from the blood to
study GPBP expression and function on these cells. For comparison, it is
necessary to study normal, healthy blood cells as well. This will be obtained
from healthy controls.

Patients will be asked to give blood during a regular outpatient clinic visit,
so it will demand no extra time investment from the patient. For control
subject, the most important burden will be time investment. A local bruise is
possible on the site of puncture.