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Jerne network theory 425 Jo-1 syndrome
Epitope on
antigen
Anti-idiotype 1
antibody
B
B
Paratope
Jerne network theory.
Jerne network theory
Niels Jerne’s hypothesis that antibodies produced in
response to a specific antigen would induce a second group
of antibodies that in turn would downregulate the original
antibody-producing cells. The second antigen (Ab-2)
would recognize epitopes of the antibody-binding region of
antibody 1. These would be anti-idiotypic antibodies that
would also be reactive with the antigen-binding regions of T
cell receptors for which they were specific. Thus, a network
of antiantibodies would produce a homeostatic effect on
the immune response to a particular antigen. This theory
was subsequently proven and confirmed by numerous
investigators.
Niels Kaj Jerne.
Jerne, Niels Kaj (1911–1994)
Immunologist born in London and educated at Leiden
and Copenhagen who shared the Nobel Prize in 1984 with
Kohler and Millstein for his contributions to immune system
theory. These include his selective theory of antibody
formation, the functional network of interacting antibodies
and lymphocytes, and distinction of self from nonself by
B
Idiotope
Anti-idiotype 2
antibody
T lymphocytes. He studied antibody synthesis and avidity,
perfected the hemolytic plaque assay, developed the natural
selection theory of antibody formation, and formulated the
idiotypic network theory. He was the director of the Paul
Ehrlich Institute in Frankfurt-am-Main, 1966, and director
of the Basel Institute for Immunology, 1969.
Complement
Agar with spleen
cells and sheep RBC
Immunized animal
Coating agar
Glass
slide
Spleen
Jerne plaque assay.
Jerne plaque assay
Refer to hemolytic plaque assay.
Jo-1 autoantibodies.
Plaques form
on glass slide
Plaqueforming
cell
Sheep
RBCs
Jo-1 autoantibodies
Thirty percent of adult patients with myositis (including
polymyositis, dermatomyositis, and combination
syndromes) form autoantibodies against the Jo-1 antigen
(histidyl-tRNA synthetase). These antibodies against Jo-1
occur in approximately 60% of patients with both myositis
and interstitial lung disease. Jo-1 antibodies are most
often found in addition to other aminoacyl synthetases in
individuals with antisynthetase syndrome, which is marked
by acute onset, steroid-responsive myositis with interstitial
lung disease, fever, symmetrical arthritis, Raynaud’s
phenomenon, and mechanic’s hands. Jo-1 autoantibodies in
patients with idiopathic polymyositis are usually associated
with severe relapse and poor prognosis.
Jo-1 syndrome
A clinical condition in which anti-Jo-1 antigen (histidyltRNA
synthetase) antibodies are produced. Arthritis,
myositis, and interstitial lung disease, may be present.
One quarter of myositis patients may manifest anti-Jo-1
antibodies.
J