Amyloid seeding of transthyretin by ex vivo cardiac fibrils and its inhibition

Saelices, Lorena; Chung, Kevin; Lee, Ji H.; Cohn, Whitaker; Whitelegge, Julian P.; Benson, Merrill D.; Eisenberg, David S.

doi:10.1073/pnas.1805131115

Title: Amyloid seeding of transthyretin by ex vivo cardiac fibrils and its inhibition

Journal Article · Thu Jun 28 00:00:00 EDT 2018 · Proceedings of the National Academy of Sciences of the United States of America

DOI:https://doi.org/10.1073/pnas.1805131115· OSTI ID:1457595

^[1]; Chung, Kevin ^[1]; Lee, Ji H. ^[1]; Cohn, Whitaker ^[2]; Whitelegge, Julian P. ^[2]; Benson, Merrill D. ^[3]; Eisenberg, David S. ^[1]

Howard Hughes Medical Institute, University of California, Los Angeles, CA 90095,, UCLA-DOE, University of California, Los Angeles, CA 90095,, Department of Biological Chemistry, University of California, Los Angeles, CA 90095,, Molecular Biology Institute, University of California, Los Angeles, CA 90095,
Neuropsychiatric Institute (NPI)-Semel Institute, University of California, Los Angeles, CA 90024,
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202

Significance Transthyretin (TTR) cardiac amyloidosis is characterized by the deposition of TTR amyloid fibrils in the heart. No therapy is currently available for wild-type cardiac amyloidosis. Hereditary cases are treated by liver transplantation, a crude form of gene therapy which replaces amyloidogenic mutant TTR by the more stable wild-type form, with the goal of halting further deposition and disease progression. However, wild-type TTR continues to deposit in the heart of many patients after the procedure. Until now, seeding of TTR fibril formation has not been demonstrated in vitro. We show that patient-extracted cardiac fibrils can seed both wild-type and mutant TTR fibril formation in vitro. This process can be inhibited by structure-based peptide inhibitors, thereby providing an alternative approach to therapy.

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Sponsoring Organization:: USDOE

Grant/Contract Number:: AC02-06CH11357

OSTI ID:: 1457595

Journal Information:: Proceedings of the National Academy of Sciences of the United States of America, Journal Name: Proceedings of the National Academy of Sciences of the United States of America Vol. 115 Journal Issue: 29; ISSN 0027-8424

Publisher:: Proceedings of the National Academy of SciencesCopyright Statement

Country of Publication:: United States

Language:: English

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Cited by: 45 works

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