A Novel Tool for Detecting Amyloid Deposits in Systemic Amyloidosis In Vitro and In Vivo

• Published in: Laboratory investigation Vol. 83; no. 12; pp. 1751 - 1759
• Main Authors: Ando, Yukio, Haraoka, Katsuki, Terazaki, Hisayasu, Tanoue, Yutaka, Ishikawa, Kensuke, Katsuragi, Shoichi, Nakamura, Masaaki, Sun, Xuguo, Nakagawa, Kazuko, Sasamoto, Kazumi, Takesako, Kazuhiro, Ishizaki, Takashi, Sasaki, Yutaka, Doh-ura, Katsumi
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01-12-2003; Nature Publishing; Nature Publishing Group
• Subjects: Adult; Amyloid - analysis; Amyloid - metabolism; Amyloid - ultrastructure; Amyloidosis; Amyloidosis - diagnosis; Amyloidosis - metabolism; Amyloidosis - pathology; Animals; Biological and medical sciences; Congo Red - chemistry; Disease Models, Animal; Female; Humans; Immunohistochemistry; In Vitro Techniques; Male; Medical sciences; Metabolic diseases; Mice; Middle Aged; Other metabolic disorders; Prealbumin - metabolism; Prealbumin - ultrastructure; Staining and Labeling - methods; Styrenes - chemistry; Styrenes - metabolism; Human; Nervous system diseases; Prion disease; Clinical form; Metabolic diseases; Exploration; Light peptide chain; Polyneuropathy; Enzymopathy; In vitro; Protein; Infection; In vivo; Autopsy; Biopsy; Amyloid protein AA; Clinical biology; Amyloidosis; Peripheral nerve disease; Technique
• ISSN: 0023-6837; 1530-0307
• DOI: 10.1097/01.LAB.0000101701.87433.C5

We synthesized (trans,trans)-1-bromo-2,5-bis-(3-hydroxycarbonyl-4-hydroxy)styrylbenzene (BSB) and used this compound to detect amyloid fibrils in autopsy and biopsy samples from patients with localized amyloidosis, such as familial prion disease, and systemic amyloidosis, such as familial amyloidotic polyneuropathy, amyloid A (AA) amyloidosis, light chain (AL) amyloidosis, and dialysis-related amyloidosis. BSB showed reactions in all Congo red-positive and immunoreactive regions of the samples examined in the study, and some amyloid fibrils in the tissues could be detected more precisely with BSB than with the other methods. In the mouse model of AA amyloidosis, injected BSB reacted with amyloid in all regions in the serial sections in which Congo red staining was positive. A highly sensitive 27-MHz quartz crystal microbalance analysis revealed that BSB showed a significant affinity for amyloid fibrils purified from familial amyloidotic polyneuropathy and dialysis-related amyloidosis samples and suppressed formation of transthyretin amyloid in vitro. These results suggest that BSB may become a valuable tool for detection of amyloid deposits in amyloidosis and of the mechanism of amyloid formation.