482
Autoimmunity in Scleroderma
Bibliography
497
Books and Reviews
497
Primary Literature
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Recent Key Primary Literature
499
Keywords
Anticentromere Antibodies
A centromere is the essential domain for proper segregation of chromosomes to
daughter cells at mitosis and meiosis. Antibodies to the centromere are found in about
20% of patients with scleroderma, almost all of whom have limited scleroderma,
formerly called
CREST
(calcinosis, Raynaud’s phenomenon, esophageal dysmotility,
sclerodactyly, and telangiectasia) syndrome.
Antitopoisomerase I Antibodies
Anti-DNA topoisomerase I antibodies, formerly termed
anti-Scl-70
antibodies,
recognize a nuclear enzyme that catalyzes the relaxation of supercoiled DNA. The
presence of antitopoisomerase I antibodies is highly speciFc to scleroderma sera.
Antitopoisomerase I antibodies identify a scleroderma subset with a high frequency of
diffuse skin thickening and pulmonary interstitial Fbrosis.
Epitope
Antigenic determinants recognized by T and/or B cells are called
immunogenic epitopes
.
Recombinant techniques of antigens can yield information crucial to uncovering T-
and B-cell epitopes. Epitopes in autoimmunity can be expanded to other epitopes on
the same protein or other proteins in the same macromolecular complexes, a
phenomenon termed
epitope spreading
.
Recombinant Protein
Large amounts of antigen can be expressed by introducing its cloned gene into
Escherichia coli
or insect cells. ±usion proteins consisting of the amino-terminal
peptides encoded by a portion of the
E. coli
β
-galactosidase or a glutathione
S-transferase peptide linked to eukaryotic proteins have been used often.
Scleroderma
Scleroderma is a multisystem disorder of unknown etiology characterized by abnormal
deposition of collagen in the skin and internal organs, and microvascular obliteration.
Circulating autoantibodies are one of the features of scleroderma.
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