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β 1H 106 bifunctional antibodies<br />

1H<br />

Refer to factor H.<br />

-2 glycoprotein-I autoantibodies<br />

Autoantibodies against β-2 glycoprotein-1 (β-2-GP1), a<br />

natural serum coagulation inhibitor. These autoantibodies<br />

represent one of the interactions of antiphospholipid<br />

antibodies with components of the clotting system.<br />

Antiphospholipid autoantibodies occur in antiphospholipid<br />

syndrome (APS), which is associated with<br />

recurrent thrombotic events, repeated fetal loss, and/<br />

or thrombocytopenia, usually associated with systemic<br />

lupus erythematosus (SLE), as well as in patients without<br />

autoimmune disease. β-2-GP1, a 50-kDa plasma<br />

protein binds to phospholipids (PLs) and lipoproteins.<br />

Binding to PLs inhibits the intrinsic blood coagulation<br />

pathway, prothrombinase activity, and ADP-dependent<br />

platelet aggregation and induces a conformational<br />

change in β-2-GP1, causing β-2-GPI autoantibodies to<br />

bind only to β-2-GP1 when it is complexed to PLs.<br />

2 microglobulin ( 2M)<br />

A thymic epithelium-derived polypeptide that is 11.8 kDa<br />

and makes up part of the major histocompatibility complex<br />

(MHC) class I molecules that appear on the surfaces of<br />

nucleated cells. It is noncovalently linked to the MHC class<br />

I polypeptide chain. It promotes maturation of T lymphocytes<br />

and serves as a chemotactic factor. β 2M makes up part<br />

of the peptide–antigen class I–β 2M complex involved in<br />

antigen presentation to cytotoxic T lymphocytes. Nascent<br />

β 2M facilitates the formation of antigenic complexes that<br />

can stimulate T lymphocytes. This monomorphic polypeptide<br />

accumulates in the serum in renal dialysis patients and<br />

may lead to β 2 microglobulin (β 2M) 2-induced amyloidosis.<br />

The extracellular protein β 2M is encoded by a nonpolymorphic<br />

gene outside the MHC. It is homologous structurally to<br />

an immunoglobulin domain and is invariant among all class<br />

I molecules.<br />

-adrenergic receptor autoantibodies<br />

Autoantibodies against β1-adrenoreceptors are<br />

detected in a significant proportion of individuals with<br />

dilated cardiomyopathy. The human heart contains<br />

both β1-adrenoreceptors and a substantial number of<br />

β2-adrenoreceptors. Both types are diminished in chronic<br />

heart failure. β1-adrenoreceptors are decreased in all<br />

types of heart failure, whereas β2-adrenoreceptors are<br />

diminished in mitral valve disease, tetralogy of Fallot, and<br />

end-stage ischemic cardiomyopathy; 30 to 40% of dilated<br />

cardiomyopathy, 22% of ischemic cardiomyopathy, and<br />

25% of alcoholic cardiomyopathy patients develop autoantibodies<br />

against β-adrenoreceptors. In familial, idiopathic,<br />

dilated cardiomyopathy, 62% of affected family members<br />

manifest the β-adrenoreceptor autoantibodies, compared<br />

to 29% in unaffected members. Dilated cardiomyopathy<br />

patients have increased frequency of HLA-DR4 antigen<br />

compared to normal persons, and 60 to 80% of patients<br />

with HLA-DR4 and dilated cardiomyopathy develop<br />

β-adrenoreceptor antibodies.<br />

beta-gamma bridge<br />

Patients with chronic liver disease such as that caused by<br />

alcohol, chronic infection, or connective tissue disease may<br />

synthesize sufficient polyclonal proteins whose electrophoretic<br />

mobilities are in the β–γ range to cause obliteration of<br />

the β and γ peaks, forming a “bridge” from one to the other.<br />

Normal<br />

Albumin Alcoholic<br />

α1 α2 β γ<br />

Beta-gamma bridge.<br />

thromboglobulin (TG)<br />

A chemokine generated through successive proteolysis of<br />

platelet basic protein (PBP), a 94-amino-acid protein present<br />

in the α granules of platelets. The plasma concentrations<br />

of both βTG and PF4 granule constituents are assayed for<br />

platelet activation in allergic states and asthma.<br />

Ernst Beutner.<br />

Beutner, Ernst<br />

A member of the Buffalo Immunology Group.<br />

bevacizumab<br />

A humanized IgG 1 monoclonal antibody that unites with<br />

vascular endothelial growth factor (VEGF) and blocks<br />

VEGF binding to its receptor, mainly on endothelial cells.<br />

It has an antiangiogenic effect, inhibiting blood vessel<br />

growth in tumors. It is used to treat patients with metastatic<br />

colorectal cancer.<br />

BFPR<br />

Abbreviation for biological false-positive reaction.<br />

biclonality<br />

In contrast to uncontrolled proliferation of a single clone<br />

of neoplastic cells which is usually associated with tumors,<br />

rarely two neoplastic cell clones may proliferate simultaneously,<br />

leading to a biclonality. For example, either neoplastic<br />

B or T lymphocytes may demonstrate this effect.<br />

bifunctional antibodies<br />

Artificially formed immunoglobulins that manifest double,<br />

well-defined antigen specificity. They are used to focus the<br />

activity of an effector cell on a target cell by their ability to<br />

bind with one combining site on the effector cell and with<br />

another on the target cell. Antibodies to helper and cytotoxic<br />

effector T cell receptors can mimic antigen and activate the T<br />

cells to proliferate, release lymphokines, or become cytotoxic.

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