Merrill Benson

  • 13806 Citations
  • 61 h-Index
1961 …2020

Research output per year

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Publications

2020
Open Access

Early data on long-term efficacy and safety of inotersen in patients with hereditary transthyretin amyloidosis: a 2-year update from the open-label extension of the NEURO-TTR trial

Brannagan, T. H., Wang, A. K., Coelho, T., Waddington Cruz, M., Polydefkis, M. J., Dyck, P. J., Plante-Bordeneuve, V., Berk, J. L., Barroso, F., Merlini, G., Conceição, I., Hughes, S. G., Kwoh, J., Jung, S. W., Guthrie, S., Pollock, M., Benson, M. D., Gertz, M., Drachman, B., Gorevic, P. & 13 others, Heitner, S., Scheinberg, M., Schmidt, H., Whelan, C., Adams, D., Campistol Plana, J. M., Gamez, J., Gane, E., Kristen, A., Obici, L., Salvi, F., Souza Bulle Oliveira, A. & Vita, G., Aug 1 2020, In : European Journal of Neurology. 27, 8, p. 1374-1381 8 p.

Research output: Contribution to journalArticle

Open Access
1 Scopus citations

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

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., Apr 1 2020, In : Journal of neurology. 267, 4, p. 1070-1079 10 p.

Research output: Contribution to journalArticle

Open Access
2 Scopus citations

Inotersen therapy of transthyretin amyloid cardiomyopathy

Dasgupta, N. R., Rissing, S. M., Smith, J., Jung, J. & Benson, M. D., Jan 2 2020, In : Amyloid. 27, 1, p. 52-58 7 p.

Research output: Contribution to journalArticle

5 Scopus citations
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. D. & 1 others, Ando, Y., Jul 19 2019, In : Journal of Biological Chemistry. 294, 29, p. 11259-11275 17 p.

Research output: Contribution to journalArticle

Open Access
1 Scopus citations

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

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. D. & Eisenberg, D. S., Apr 12 2019, In : Journal of Biological Chemistry. 294, 15, p. 6130-6141 12 p.

Research output: Contribution to journalArticle

Open Access
4 Scopus citations

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. D. & Kalopissis, A. D., Sep 2019, In : Kidney international. 96, 3, p. 628-641 14 p.

Research output: Contribution to journalArticle

7 Scopus citations

Inotersen treatment for ATTR amyloidosis

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

Research output: Contribution to journalLetter

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

2018

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

Benson, M. D., Buxbaum, J. N., Eisenberg, D. S., Merlini, G., Saraiva, M. J. M., Sekijima, Y., Sipe, J. D. & Westermark, P., Oct 2 2018, In : Amyloid. 25, 4, p. 215-219 5 p.

Research output: Contribution to journalArticle

124 Scopus citations

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. D. & 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

10 Scopus citations

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. D. & Gertz, M. A., Jul 3 2018, In : Amyloid. 25, 3, p. 180-188 9 p.

Research output: Contribution to journalArticle

8 Scopus citations

Inotersen treatment for patients with Hereditary transthyretin amyloidosis

Benson, M. D., 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

325 Scopus citations
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

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

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

Dyck, P. J., Kincaid, J. C., 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. D., 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., Nov 2017, In : Muscle and Nerve. 56, 5, p. 901-911 11 p.

Research output: Contribution to journalArticle

27 Scopus citations

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

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

32 Scopus citations

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

2 Scopus citations

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

8 Scopus citations
2016

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

4 Scopus citations

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

251 Scopus citations

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

1 Scopus citations

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

7 Scopus citations

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

3 Scopus citations

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

Fix, O. K., Stock, P. G., Lee, B. K. & Benson, M. D., Apr 2 2016, In : Amyloid. 23, 2, p. 132-133 2 p.

Research output: Contribution to journalLetter

3 Scopus citations

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

62 Scopus citations
2015

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

38 Scopus citations

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

158 Scopus citations

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

7 Scopus citations
2014

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

Sipe, J. D., Benson, M. D., 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 journalReview article

313 Scopus citations

Peripheral Neuropathies

Scherer, S. S., Kleopa, K. A. & Benson, M. D., 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

3 Scopus citations

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

Suanprasert, N., Berk, J. L., Benson, M. D., 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

45 Scopus citations

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

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

Research output: Contribution to journalArticle

5 Scopus citations
2013

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

Ihse, E., Rapezzi, C., Merlini, G., Benson, M. D., 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

54 Scopus citations

Amyloidosis and Other Protein Deposition Diseases

Benson, M. D., Aug 29 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

1 Scopus citations

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 1 2013, In : Amyloid. 20, 1, p. 52-55 4 p.

Research output: Contribution to journalArticle

2 Scopus citations

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. D. & Kluve-Beckerman, B., Jun 2013, In : Amyloid. 20, 2, p. 61-71 11 p.

Research output: Contribution to journalArticle

5 Scopus citations

Liver transplantation and transthyretin amyloidosis

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

Research output: Contribution to journalReview article

58 Scopus citations
2012

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

Sipe, J. D., Benson, M. D., Buxbaum, J. N., Ikeda, S. I., Merlini, G., Saraiva, M. J. M. & Westermark, P., Dec 1 2012, In : Amyloid. 19, 4, p. 167-170 4 p.

Research output: Contribution to journalArticle

184 Scopus citations

Amyloidosis of the gastrointestinal tract and liver

Cummings, O. W. & Benson, M. D., 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

2 Scopus citations

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 1 2012, In : Amyloid. 19, SUPPL. 1, p. 43-44 2 p.

Research output: Contribution to journalArticle

108 Scopus citations

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

1 Scopus citations

Pathogenesis of transthyretin amyloidosis

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

Research output: Contribution to journalArticle

28 Scopus citations

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

7 Scopus citations

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

7 Scopus citations