Contents of Kit
1. Polystyrene microwell ELISA plates coated with a purified antigen(12-1 x 8 wells), with holder in foil package containing desiccants
2. Negative Control, 1 vial of buffer 1.2 mL
3. Positive Control, 1 vial of buffer 1.2 mL
4. Calibrator A, 1 vial of buffer containing preservative, prediluted, 1.2 mL
5. Calibrator B, 1 vial of buffer containing preservative, prediluted, 1.2 mL
6. Calibrator C, 1 vial of buffer containing preservative, prediluted, 1.2 mL
7. Calibrator D, 1 vial of buffer containing preservative, prediluted, 1.2 mL
8. Sample Diluent, 1 vial – colored straw containing PBS-buffered saline, protein stabilizers and preservative, 50 mL.
9. Antibody Enzyme Conjugate, colored blue containing buffer, protein stabilizers and preservative, 12 mL
10. Wash Buffer (20 ×), 50 mL
11. TMB Chromogen, containing stabilizers, 10 mL
12. Stop Solution, Colorless, 10 mL
General Description
Progressive systemic scleroderma (PSS) is an autoimmune multisystemic disorder characterized by tight skin. PSS is a disorder of the vascular connective tissue leading to slowly progressive fibrosis and to sclerosis in the advanced phases of the disease. Aside from skin the gastrointestinal tract of the patients is the most affected organ. Moreover kidney, lung, heart and muscles are involved.
Serologically PSS can be characterized by the detection of antinuclear antibodies. Up to 86% of the patients suffering from scleroderma exhibit autoantibodies against the Scl-70 antigen, i.e. Topoisomerase I. Anti-Scl-70 antibodies are mostly present in patients with the more diffuse manifestations of PSS (with lungs and joints being affected and fast progression tendency). CREST-Syndrome is a variant of PSS with a more protracted course of disease. However, prognosis is much better compared to patients exclusively suffering from PSS. The acronym CREST has been derived from the first letters of the five most important clinical manifestations: Calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, teleangiectasia). In patients suffering from CREST syndrome circulating antibodies against the centromere protein B can be detected. Up to 70% of all CREST patients exhibit Anti-Centromere B autoantibodies. The determination of Anti-Centromere B autoantibodies is of prognostic significance in diagnosing Raynaud's phenomenon. Raynaud's phenomenon often comes out as the first symptom of scleroderma and precedes the additional manifestations by several years. Also in patients suffering from primary biliary cirrhosis (PBC) Anti-Centromere B antibodies can be found. PBC and CREST often overlap. Anti-Centromere B are found in about 10-20% of sera from patients with PBC preferentially identifying those patients with Raynaud's phenomenon and sclerodactyly. Along with other centromere proteins (e.g. CENP-A (19 kDa), CENPC (140 kDa)) centromere protein B (80 kDa) belongs to a protein complex of the chromosomal kinetochore.
COA
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