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Proteinase 3 Antibody and Anti-Double-Stranded DNA in a Patient With Immunoglobin Light Chain Amyloidosis

Jessica K Cobb1, Lachlan Shiver1, Charles R Russell2,3

  • 1Nephrology, University of South Florida Morsani College of Medicine, Tampa, USA.

Cureus
|November 29, 2023
PubMed
Summary

This case report details a patient with renal amyloidosis who unexpectedly tested positive for both Proteinase 3 (PR3) anti-neutrophil cytoplasmic antibodies (ANCA) and anti-double-stranded DNA (anti-dsDNA) antibodies, a rare autoimmune presentation.

Keywords:
al amyloidosisantineutrophil cytoplasmic antibody (anca)dsdnampo-ancapr3

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Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...