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2020

Editorial Commentary: Advances in transthyretin amyloid cardiomyopathy

Benson, M. D. & Dasgupta, N. R., Jan 1 2020, (Accepted/In press) In : Trends in Cardiovascular Medicine.

Research output: Contribution to journalEditorial

2019

A cell-based high-throughput screening method to directly examine transthyretin amyloid fibril formation at neutral pH

Ueda, M., Okada, M., Mizuguchi, M., Kluve-Beckerman, B., Kanenawa, K., Isoguchi, A., Misumi, Y., Tasaki, M., Ueda, A., Kanai, A., Sasaki, R., Masuda, T., Inoue, Y., Nomura, T., Shinriki, S., Shuto, X. T., Kai, H., Yamashita, T., Matsui, H., Benson, M. & 1 others, Ando, Y., Jan 1 2019, In : Journal of Biological Chemistry. 294, 29, p. 11259-11275 17 p.

Research output: Contribution to journalArticle

Open Access
High-Throughput Screening Assays
Prealbumin
Amyloid
Screening
Throughput

ALECT2 and NASH

Cummings, O., Dasgupta, N. & Benson, M., Mar 29 2019, In : Amyloid. 26, sup1, p. 158-159 2 p.

Research output: Contribution to journalLetter

3 Citations (Scopus)

A pair of peptides inhibits seeding of the hormone transporter transthyretin into amyloid fibrils

Saelices, L., Nguyen, B. A., Chung, K., Wang, Y., Ortega, A., Lee, J. H., Coelho, T., Bijzet, J., Benson, M. & Eisenberg, D. S., Jan 1 2019, In : Journal of Biological Chemistry. 294, 15, p. 6130-6141 12 p.

Research output: Contribution to journalArticle

Open Access
Prealbumin
Amyloid
Hormones
Peptides
Familial Amyloidosis

A transgenic mouse model reproduces human hereditary systemic amyloidosis

Chabert, M., Rousset, X., Colombat, M., Lacasa, M., Kakanakou, H., Bourderioux, M., Brousset, P., Burlet-Schiltz, O., Liepnieks, J. J., Kluve-Beckerman, B., Lambert, G., Châtelet, F. P., Benson, M. & Kalopissis, A. D., Jan 1 2019, In : Kidney international.

Research output: Contribution to journalArticle

Familial Amyloidosis
Transgenic Mice
Amyloidosis
Amyloid
Amyloidogenic Proteins
5 Citations (Scopus)

Inotersen (transthyretin-specific antisense oligonucleotide) for treatment of transthyretin amyloidosis

Benson, M., Dasgupta, N. & Monia, B. P., Feb 1 2019, In : Neurodegenerative disease management. 9, 1, p. 25-30 6 p.

Research output: Contribution to journalArticle

Prealbumin
Antisense Oligonucleotides
Peripheral Nervous System Diseases
Familial Amyloidosis
Quality of Life

Inotersen preserves or improves quality of life in hereditary transthyretin amyloidosis

Coelho, T., Yarlas, A., Waddington-Cruz, M., White, M. K., Sikora Kessler, A., Lovley, A., Pollock, M., Guthrie, S., Ackermann, E. J., Hughes, S. G., Karam, C., Khella, S., Gertz, M., Merlini, G., Obici, L., Schmidt, H. H., Polydefkis, M., Dyck, P. J. B., Brannagan, T. H., Conceição, I. & 2 others, Benson, M. D. & Berk, J. L., Jan 1 2019, (Accepted/In press) In : Journal of Neurology.

Research output: Contribution to journalArticle

Open Access
Familial Amyloidosis
Quality of Life
Placebos
Polyneuropathies
Diabetic Neuropathies

Inotersen therapy of transthyretin amyloid cardiomyopathy

Dasgupta, N. R., Rissing, S. M., Smith, J., Jung, J. & Benson, M. D., Jan 1 2019, (Accepted/In press) In : Amyloid.

Research output: Contribution to journalArticle

Prealbumin
Cardiomyopathies
Amyloid
Therapeutics
Safety

Inotersen treatment for ATTR amyloidosis

Benson, M., Mar 29 2019, In : Amyloid. 26, sup1, p. 27-28 2 p.

Research output: Contribution to journalLetter

Amyloidosis

Treatment of ATTR cardiomyopathy with a TTR specific antisense oligonucleotide, inotersen

Dasgupta, N. R. & Benson, M. D., Mar 29 2019, In : Amyloid. 26, sup1, p. 20-21 2 p.

Research output: Contribution to journalLetter

Antisense Oligonucleotides
Cardiomyopathies
2018
51 Citations (Scopus)

Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee

Benson, M., Buxbaum, J. N., Eisenberg, D. S., Merlini, G., Saraiva, M. J. M., Sekijima, Y., Sipe, J. D. & Westermark, P., Jan 1 2018, (Accepted/In press) In : Amyloid.

Research output: Contribution to journalArticle

Amyloidosis
Amyloid
Terminology
Serum Amyloid P-Component
Birefringence
6 Citations (Scopus)

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

Saelices, L., Chung, K., Lee, J. H., Cohn, W., Whitelegge, J. P., Benson, M. & Eisenberg, D. S., Jul 17 2018, In : Proceedings of the National Academy of Sciences of the United States of America. 115, 29, p. E6741-E6750

Research output: Contribution to journalArticle

Prealbumin
Amyloid
Seeds
Protein Precursors
Liver
5 Citations (Scopus)

Hereditary transthyretin amyloidosis: baseline characteristics of patients in the NEURO-TTR trial

Waddington-Cruz, M., Ackermann, E. J., Polydefkis, M., Heitner, S. B., Dyck, P. J., Barroso, F. A., Wang, A. K., Berk, J. L., Dyck, P. J. B., Monia, B. P., Hughes, S. G., Tai, L., Jesse Kwoh, T., Jung, S. W., Coelho, T., Benson, M. & Gertz, M. A., Jan 1 2018, (Accepted/In press) In : Amyloid.

Research output: Contribution to journalArticle

Familial Amyloidosis
Amyloidosis
Polyneuropathies
Quality of Life
Cardiomyopathies
199 Citations (Scopus)

Inotersen treatment for patients with Hereditary transthyretin amyloidosis

Benson, M., Waddington-Cruz, M., Berk, J. L., Polydefkis, M., Dyck, P. J., Wang, A. K., Planté-Bordeneuve, V., Barroso, F. A., Merlini, G., Obici, L., Scheinberg, M., Brannagan, T. H., Litchy, W. J., Whelan, C., Drachman, B. M., Adams, D., Heitner, S. B., Conceição, I., Schmidt, H. H., Vita, G. & 15 others, Campistol, J. M., Gamez, J., Gorevic, P. D., Gane, E., Shah, A. M., Solomon, S. D., Monia, B. P., Hughes, S. G., Jesse Kwoh, T., McEvoy, B. W., Jung, S. W., Baker, B. F., Ackermann, E. J., Gertz, M. A. & Coelho, T., Jul 5 2018, In : New England Journal of Medicine. 379, 1, p. 22-31 10 p.

Research output: Contribution to journalArticle

Familial Amyloidosis
Prealbumin
Placebos
Quality of Life
Therapeutics
2017

AA cardiomyopathy

Dasgupta, N., Cummings, O., Phillips, C. & Benson, M., Mar 16 2017, In : Amyloid. 24, p. 138-139 2 p.

Research output: Contribution to journalLetter

Cardiomyopathies

An aggressive form of transthyretin amyloidosis

Dasgupta, N., Wang, A. K., Hardwick, J. & Benson, M., Mar 16 2017, In : Amyloid. 24, p. 95-96 2 p.

Research output: Contribution to journalLetter

Amyloidosis, Hereditary, Transthyretin-Related
22 Citations (Scopus)

Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial

Dyck, P. J., Kincaid, J., Dyck, P. J. B., Chaudhry, V., Goyal, N. A., Alves, C., Salhi, H., Wiesman, J. F., Labeyrie, C., Robinson-Papp, J., Cardoso, M., Laura, M., Ruzhansky, K., Cortese, A., Brannagan, T. H., Khoury, J., Khella, S., Waddington-cruz, M., Ferreira, J., Wang, A. K. & 24 others, Pinto, M. V., Ayache, S. S., Benson, M., Berk, J. L., Coelho, T., Polydefkis, M., Gorevic, P., Adams, D. H., Plante-Bordeneuve, V., Whelan, C., Merlini, G., Heitner, S., Drachman, B. M., Conceição, I., Klein, C. J., Gertz, M. A., Ackermann, E. J., Hughes, S. G., Mauermann, M. L., Bergemann, R., Lodermeier, K. A., Davies, J. L., Carter, R. E. & Litchy, W. J., 2017, (Accepted/In press) In : Muscle and Nerve.

Research output: Contribution to journalArticle

Polyneuropathies
Health
Neurophysiology
Neurologic Manifestations
Oligonucleotides

Familial wild-type transthyretin cardiomyopathy

Benson, M., Berk, J. L., Connors, L. H. & Dasgupta, N., Mar 16 2017, In : Amyloid. 24, p. 91-92 2 p.

Research output: Contribution to journalLetter

Prealbumin
Cardiomyopathies

Insulin: a diagnostic warning

Liepnieks, J. J., Benson, M. & Kluve-Beckerman, B., Mar 16 2017, In : Amyloid. 24, p. 159-160 2 p.

Research output: Contribution to journalLetter

Insulin
21 Citations (Scopus)

Safety and efficacy of a TTR specific antisense oligonucleotide in patients with transthyretin amyloid cardiomyopathy

Benson, M., Dasgupta, N., Rissing, S., Smith, J. & Feigenbaum, H., Sep 13 2017, (Accepted/In press) In : Amyloid. p. 1-7 7 p.

Research output: Contribution to journalArticle

Prealbumin
Antisense Oligonucleotides
Cardiomyopathies
Amyloid
Safety
1 Citation (Scopus)

Transthyretin amyloidosis: a little history of hereditary amyloidosis

Benson, M., Mar 16 2017, In : Amyloid. 24, p. 76-77 2 p.

Research output: Contribution to journalLetter

Familial Amyloidosis
Amyloidosis, Hereditary, Transthyretin-Related
7 Citations (Scopus)

Treatment of transthyretin cardiomyopathy with a TTR-specific antisense oligonucleotide (IONIS-TTRRx)

Benson, M., Ackermann, E. J. & Monia, B. P., Mar 16 2017, In : Amyloid. 24, p. 134-135 2 p.

Research output: Contribution to journalLetter

Amyloid Neuropathies
Familial Amyloid Neuropathies
Prealbumin
Antisense Oligonucleotides
Cardiomyopathies
2016
3 Citations (Scopus)

Amyloid Cardiomyopathy

Benson, M. & Dasgupta, N., Jul 5 2016, In : Journal of the American College of Cardiology. 68, 1, p. 25-28 4 p.

Research output: Contribution to journalComment/debate

Prealbumin
Amyloidosis
Cardiomyopathies
Amyloid
Immunohistochemistry
210 Citations (Scopus)

Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification International Society of Amyloidosis 2016 Nomenclature Guidelines

Sipe, J. D., Benson, M., Buxbaum, J. N., Ikeda, S. I., Merlini, G., Saraiva, M. J. M. & Westermark, P., Oct 1 2016, In : Amyloid. 23, 4, p. 209-213 5 p.

Research output: Contribution to journalArticle

Amyloidogenic Proteins
Amyloidosis
Amyloid
Terminology
Guidelines
1 Citation (Scopus)

Biochemical characterization of vitreous amyloid formed after liver transplantation

Liepnieks, J. J., Phan, A-D., Wise, R. J., Hrisomalos, F. N. & Benson, M., Apr 1 2016, (Accepted/In press) In : Amyloid. p. 1-2 2 p.

Research output: Contribution to journalArticle

Familial Amyloid Neuropathies
Amyloidogenic Proteins
Vitreous Body
Prealbumin
Retinal Pigment Epithelium
3 Citations (Scopus)

Carbamylation of the amino-terminal residue (Gly1) of mouse serum amyloid A promotes amyloid formation in a cell culture model

Kluve-Beckerman, B., Liepnieks, J. J., Benson, M., Lai, X., Qi, G. & Wang, M., Dec 1 2016, In : FEBS Letters. 590, 23, p. 4296-4307 12 p.

Research output: Contribution to journalLetter

Serum Amyloid A Protein
Cell culture
Amyloid
Cell Culture Techniques
Amyloidosis
2 Citations (Scopus)

Disease-free survival following high dose or standard dose therapy in patients with amyloidosis

Kiel, P. J., Trueg, A. O., Ferguson, M., Benson, M. & Abonour, R., Jul 1 2016, In : British Journal of Haematology. 174, 1, p. 153-155 3 p.

Research output: Contribution to journalLetter

Melphalan
Amyloidosis
Disease-Free Survival
Therapeutics
Bortezomib
2 Citations (Scopus)

Liver transplant alone without kidney transplant for fibrinogen Aα-chain (AFib) renal amyloidosis

Fix, O. K., Stock, P. G., Lee, B. K. & Benson, M., Mar 10 2016, (Accepted/In press) In : Amyloid. p. 1-2 2 p.

Research output: Contribution to journalArticle

49 Citations (Scopus)

Suppressing transthyretin production in mice, monkeys and humans using 2nd-Generation antisense oligonucleotides

Ackermann, E. J., Guo, S., Benson, M., Booten, S., Freier, S., Hughes, S. G., Kim, T. W., Jesse Kwoh, T., Matson, J., Norris, D., Yu, R., Watt, A. & Monia, B. P., Jun 17 2016, (Accepted/In press) In : Amyloid. p. 1-10 10 p.

Research output: Contribution to journalArticle

Prealbumin
Antisense Oligonucleotides
Haplorhini
Amyloidosis
Blood Proteins
2015
29 Citations (Scopus)

A Review of Tafamidis for the Treatment of Transthyretin-Related Amyloidosis

Waddington Cruz, M. & Benson, M., Dec 1 2015, In : Neurology and Therapy. 4, 2, p. 61-79 19 p.

Research output: Contribution to journalReview article

Prealbumin
Therapeutics
Safety
Disease Progression
Benzoxazoles
122 Citations (Scopus)

Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis

Gertz, M. A., Benson, M., Dyck, P. J., Grogan, M., Coelho, T., Cruz, M., Berk, J. L., Plante-Bordeneuve, V., Schmidt, H. H. J. & Merlini, G., Dec 1 2015, In : Journal of the American College of Cardiology. 66, 21, p. 2451-2466 16 p.

Research output: Contribution to journalArticle

Amyloidosis
Cardiomyopathies
Therapeutics
Amyloidosis, Hereditary, Transthyretin-Related
6 Citations (Scopus)

Hereditary systemic immunoglobulin light-chain amyloidosis

Benson, M., Liepnieks, J. J. & Kluve-Beckerman, B., 2015, In : Blood. 125, 21, p. 3281-3286 6 p.

Research output: Contribution to journalArticle

Immunoglobulin Light Chains
Amyloidosis
Amyloid
Mutation
Genes
2014
297 Citations (Scopus)

Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis

Sipe, J. D., Benson, M., Buxbaum, J. N., Ikeda, S. I., Merlini, G., Saraiva, M. J. M. & Westermark, P., Dec 1 2014, In : Amyloid. 21, 4, p. 221-224 4 p.

Research output: Contribution to journalArticle

Amyloidogenic Proteins
Amyloidosis
Amyloid
Terminology
3 Citations (Scopus)

Peripheral Neuropathies

Scherer, S. S., Kleopa, K. A. & Benson, M., Nov 13 2014, Rosenberg's Molecular and Genetic Basis of Neurological and Psychiatric Disease: Fifth Edition. Elsevier Inc., p. 1051-1074 24 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Peripheral Nervous System Diseases
Genes
Mutation
Schwann Cells
Nervous System Diseases
38 Citations (Scopus)

Retrospective study of a TTR FAP cohort to modify NIS + 7 for therapeutic trials

Suanprasert, N., Berk, J. L., Benson, M., Dyck, P. J. B., Klein, C. J., Gollob, J. A., Bettencourt, B. R., Karsten, V. & Dyck, P. J., Sep 15 2014, In : Journal of the Neurological Sciences. 344, 1-2, p. 121-128 8 p.

Research output: Contribution to journalArticle

Ulnar Nerve
Retrospective Studies
Neural Conduction
Action Potentials
Amyloid Neuropathies
5 Citations (Scopus)

The pathologic spectrum of oculoleptomeningeal amyloidosis with Val30Gly transthyretin gene mutation in a postmortem case

Martin, S. E., Benson, M. & Hattab, E. M., 2014, In : Human Pathology. 45, 5, p. 1105-1108 4 p.

Research output: Contribution to journalArticle

Prealbumin
Amyloidosis
Amyloid
Familial Amyloidosis
Mutation
2013
48 Citations (Scopus)

Amyloid fibrils containing fragmented ATTR may be the standard fibril composition in ATTR amyloidosis

Ihse, E., Rapezzi, C., Merlini, G., Benson, M., Ando, Y., Suhr, O. B., Ikeda, S. I., Lavatelli, F., Obici, L., Quarta, C. C., Leone, O., Jono, H., Ueda, M., Lorenzini, M., Liepnieks, J., Ohshima, T., Tasaki, M., Yamashita, T. & Westermark, P., Sep 2013, In : Amyloid. 20, 3, p. 142-150 9 p.

Research output: Contribution to journalArticle

Amyloidosis
Amyloid
Familial Amyloidosis
Phenotype
Cardiomyopathies
1 Citation (Scopus)

Amyloidosis and Other Protein Deposition Diseases

Benson, M., 2013, Emery and Rimoin's Principles and Practice of Medical Genetics. Elsevier Ltd, p. 1-18 18 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Amyloidosis
Proteins
2 Citations (Scopus)

Fibrinogen A alpha-chain amyloidosis: Report of the first case in Latin America

Machado, J. R., Silva, M. V. D., Neves, P. D. M. D. M., Oliveira, F. A. D., Corrêa, R. R. M., Rodrigues, W. V. D., Benson, M. & Reis, M. A. D., Mar 2013, In : Amyloid. 20, 1, p. 52-55 4 p.

Research output: Contribution to journalArticle

Latin America
Amyloidosis
Fibrinogen
Familial Amyloidosis
Birefringence
5 Citations (Scopus)

Human SAA1-derived amyloid deposition in cell culture: A consistent model utilizing human peripheral blood mononuclear cells and serum-free medium

Ishii, W., Liepnieks, J. J., Yamada, T., Benson, M. & Kluve-Beckerman, B., Jun 2013, In : Amyloid. 20, 2, p. 61-71 11 p.

Research output: Contribution to journalArticle

Serum-Free Culture Media
Amyloid
Blood Cells
Cell Culture Techniques
Serum
55 Citations (Scopus)

Liver transplantation and transthyretin amyloidosis

Benson, M., Feb 2013, In : Muscle and Nerve. 47, 2, p. 157-162 6 p.

Research output: Contribution to journalArticle

Prealbumin
Liver Transplantation
Polyneuropathies
Amyloid
Life Support Care
2012
179 Citations (Scopus)

Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis

Sipe, J. D., Benson, M., Buxbaum, J. N., Ikeda, S. I., Merlini, G., Saraiva, M. J. M. & Westermark, P., Dec 2012, In : Amyloid. 19, 4, p. 167-170 4 p.

Research output: Contribution to journalArticle

Amyloidogenic Proteins
Amyloidosis
Amyloid
Terminology
Congo Red
2 Citations (Scopus)

Amyloidosis of the gastrointestinal tract and liver

Cummings, O. & Benson, M., Jan 1 2012, Amyloid and Related Disorders: Surgical Pathology and Clinical Correlations. Humana Press Inc., p. 339-360 22 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Amyloidosis
Amyloid
Gastrointestinal Tract
Pathology
Liver
106 Citations (Scopus)

Clinical development of an antisense therapy for the treatment of transthyretin-associated polyneuropathy

Ackermann, E. J., Guo, S., Booten, S., Alvarado, L., Benson, M., Hughes, S. & Monia, B. P., Jun 2012, In : Amyloid. 19, SUPPL. 1, p. 43-44 2 p.

Research output: Contribution to journalArticle

Prealbumin
Polyneuropathies
Familial Amyloid Neuropathies
Amyloid
Therapeutics
1 Citation (Scopus)

Emerging therapies for amyloidosis

Benson, M., Jan 1 2012, Amyloid and Related Disorders: Surgical Pathology and Clinical Correlations. Humana Press Inc., p. 393-397 5 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Antisense Oligonucleotides
Amyloidosis
Liver Transplantation
Small Interfering RNA
Therapeutics
26 Citations (Scopus)

Pathogenesis of transthyretin amyloidosis

Benson, M., Jun 2012, In : Amyloid. 19, SUPPL. 1, p. 14-15 2 p.

Research output: Contribution to journalArticle

Prealbumin
Proteolysis
Mutation
Choroid
Amyloidosis, Hereditary, Transthyretin-Related
7 Citations (Scopus)

Solid organ transplantation for non-TTR hereditary amyloidosis: Report from the 1st International Workshop on the Hereditary Renal Amyloidoses

Stangou, A. J., Lobato, L., Zeldenrust, S., Rela, M., Portmann, B., Linke, R. P., Conceicao, I., Otto, G., Wilczek, H., Suhr, O., Azoulay, D., Grateau, G., Picken, M., O'Grady, J., Heaton, N., Ericzon, B. G. & Benson, M. D., Jun 2012, In : Amyloid. 19, SUPPL. 1, p. 81-84 4 p.

Research output: Contribution to journalArticle

Familial Amyloidosis
Organ Transplantation
Amyloidosis
Amyloidogenic Proteins
Kidney
7 Citations (Scopus)

The hereditary amyloidoses

Benson, M., Jan 1 2012, Amyloid and Related Disorders: Surgical Pathology and Clinical Correlations. Humana Press Inc., p. 53-67 15 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Familial Amyloidosis
Gelsolin
Apolipoprotein A-II
Cystatin C
Prealbumin
1 Citation (Scopus)

κ I light chain AL amyloidosis presenting with rapidly progressive renal and hepatic failure with unusual renal amyloid distribution

Oe, Y., Nakaya, I., Yahata, M., Murata, O., Yaegashi, H., Sakuma, T., Sato, H., Liepnieks, J. J., Benson, M. & Soma, J., Jan 2012, In : Clinical Nephrology. 77, 1, p. 66-70 5 p.

Research output: Contribution to journalArticle

Liver Failure
Amyloidosis
Amyloid
Renal Insufficiency
Intrahepatic Cholestasis
2011
2 Citations (Scopus)

AA amyloidosis: Potential therapy with antisense oligonucleotides

Kluve-Beckerman, B., Hardwick, J., Du, L. & Benson, M., Jun 2011, In : Amyloid. 18, SUPPL. 1, p. 200-202 3 p.

Research output: Contribution to journalArticle

Antisense Oligonucleotides
Amyloidosis
Amyloid
Serum Amyloid A Protein
Therapeutics