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Untitled - D Ank Unlimited

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polymeric Ig 583 polymyositis (PM)<br />

the original DNA segment is transformed into millions<br />

of copies. PCR methodology has been used for multiple<br />

purposes including detection of human immunodeficiency<br />

virus 1 (HIV-1), prenatal diagnosis of sickle cell anemia,<br />

and gene rearrangements in lymphoproliferative disorders<br />

among other applications. The technique is used principally<br />

to prepare enough DNA for analysis by available<br />

methods and is used widely in diagnostic work. PCR has a<br />

99.99% sensitivity.<br />

polymeric Ig<br />

Immunoglobulin molecules comprised of numerous identical<br />

H 2L 2 monomers linked by a J chain. Pentameric IgM<br />

[(H 2L 2)5], with ten antigen-binding sites and dimeric IgA<br />

[(H 2L 2)2] with four, are examples.<br />

polymers<br />

Molecules composed of more than one repeating unit;<br />

immunoglobulins composed of more than one basic<br />

monomeric four-polypeptide chain unit. IgAs may exist as<br />

dimers with two units or as multimers. The IgM molecule is<br />

pentameric, containing five monomeric units.<br />

polymorphism<br />

The occurrence of different variants of a gene or trait in<br />

a population. The presence of two or more forms such as<br />

ABO and Rh blood groups, in individuals of the same<br />

species. This is due to two or more variants at a certain<br />

genetic locus occurring with considerable frequency in a<br />

population. Polymorphisms are also expressed in the human<br />

leukocyte antigen (HLA) system, as well as in allotypes of<br />

immunoglobulin γ and κ chains. Genetic polymorphism<br />

refers to the presence of two or more alleles of a given gene<br />

within a population with the variant alleles each appearing<br />

at a frequency >1%.<br />

9–15 µm<br />

Schematic representation of a polymorphonuclear leukocyte (PMN).<br />

polymorphonuclear leukocyte (PMN)<br />

White blood cells with lobulated nuclei that are often<br />

trilobed. PMNs are of myeloid cell lineage and in mature<br />

form may be differentiated into neutrophils, eosinophils,<br />

and basophils. This distinction is based on the staining<br />

characteristics of their cytoplasmic specific or secondary<br />

granules. PMNs measure approximately 13 μm in diameter<br />

and are active in acute inflammatory responses.<br />

polymyositis (PM)<br />

An acute or chronic inflammatory disease of muscle,<br />

involving fibers of the voluntary muscles; it is twice as<br />

common in women as in men. Lymphocytes in polymyositis<br />

Substances Associated with Neutrophils<br />

Azurophil Granules<br />

(Primary Granules)<br />

Specific Granules<br />

(Secondary Granules)<br />

Bacterial permeabilityinducing<br />

protein (BPI)<br />

Cathepsin G<br />

Cationic antimicrobial<br />

protein (CAP) 57<br />

Cationic antimicrobial<br />

protein (CAP) 37<br />

Defensins:<br />

HP1<br />

HP2<br />

HP3<br />

Elastase<br />

Lysozyme<br />

Myeloperoxidase Bacterial chemotaxin receptors<br />

Collagenase<br />

C5a receptors<br />

Gelatinase<br />

Lactoferrin<br />

Lysozyme<br />

NADPH<br />

Vitamin B 12-binding protein<br />

Polymorphonuclear leukocyte (PMN).<br />

subjects produce a cytotoxin when incubated with autologous<br />

muscle. Biopsies of involved muscle reveal infiltration<br />

by lymphocytes and plasma cells. PM characteristically<br />

manifests autoantibodies against aminoacyl tRNA<br />

synthetases. Antibodies can be demonstrated against the<br />

nuclear antigens Jo-1, PM-Scl, and RNP. Patients may<br />

develop polyclonal hypergammaglobulinemia. One fifth of<br />

patients may develop rheumatoid factors and antinuclear<br />

antibodies. Cellular immunity appears important in the<br />

pathogenesis, as exemplified by lymphocytes of patients<br />

with polymyositis responding to their own muscle antigens<br />

as if they were alien. Patients often complain of muscle<br />

weakness, especially in the proximal muscles of extremities.<br />

To diagnose polymyositis, a minimum of three of the<br />

following must be present: (1) shoulder or pelvic girdle<br />

weakness, (2) myositis as revealed by biopsy, (3) increased<br />

P

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