Literature DB >> 34850359

Current and Emerging Therapies for Hereditary Transthyretin Amyloidosis: Strides Towards a Brighter Future.

Laura Obici1, Roberta Mussinelli2.   

Abstract

The past few years have witnessed an unprecedented acceleration in the clinical development of novel therapeutic options for hereditary transthyretin amyloidosis. Recently approved agents and drugs currently under investigation not only represent a major breakthrough in this field but also provide validation of the therapeutic potential of innovative approaches, like RNA interference and CRISPR-Cas9-mediated gene editing, in rare inherited disorders. In this review, we describe the evolving therapeutic landscape for hereditary transthyretin amyloidosis and discuss how this highly disabling and fatal condition is turning into a treatable disease. We also provide an overview of the molecular mechanisms involved in transthyretin (TTR) amyloid formation and regression, to highlight how a deeper understanding of these processes has contributed to the identification of novel treatment targets. Finally, we focus on major areas of uncertainty and unmet needs that deserve further efforts to improve long-term patients' outcomes and allow for a brighter future.
© 2021. The American Society for Experimental NeuroTherapeutics, Inc.

Entities:  

Keywords:  Amyloidosis; Gene-silencing; Peripheral neuropathy; Stabilizers; Transthyretin

Mesh:

Substances:

Year:  2021        PMID: 34850359      PMCID: PMC8804119          DOI: 10.1007/s13311-021-01154-y

Source DB:  PubMed          Journal:  Neurotherapeutics        ISSN: 1878-7479            Impact factor:   6.088


  119 in total

1.  A peculiar form of peripheral neuropathy; familiar atypical generalized amyloidosis with special involvement of the peripheral nerves.

Authors:  C ANDRADE
Journal:  Brain       Date:  1952-09       Impact factor: 13.501

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

Authors:  Lorena Saelices; Kevin Chung; Ji H Lee; Whitaker Cohn; Julian P Whitelegge; Merrill D Benson; David S Eisenberg
Journal:  Proc Natl Acad Sci U S A       Date:  2018-06-28       Impact factor: 11.205

3.  New effective treatment options reinforce disease awareness: the case of transthyretin cardiac amyloidosis.

Authors:  Stefano Perlini; Roberta Mussinelli; Francesco Salinaro
Journal:  Eur J Heart Fail       Date:  2021-02-11       Impact factor: 15.534

4.  Transthyretin Stabilization by AG10 in Symptomatic Transthyretin Amyloid Cardiomyopathy.

Authors:  Daniel P Judge; Stephen B Heitner; Rodney H Falk; Mathew S Maurer; Sanjiv J Shah; Ronald M Witteles; Martha Grogan; Van N Selby; Daniel Jacoby; Mazen Hanna; Jose Nativi-Nicolau; Jignesh Patel; Satish Rao; Uma Sinha; Cameron W Turtle; Jonathan C Fox
Journal:  J Am Coll Cardiol       Date:  2019-03-15       Impact factor: 24.094

5.  Monitoring effectiveness and safety of Tafamidis in transthyretin amyloidosis in Italy: a longitudinal multicenter study in a non-endemic area.

Authors:  A Cortese; G Vita; M Luigetti; M Russo; G Bisogni; M Sabatelli; F Manganelli; L Santoro; T Cavallaro; G M Fabrizi; A Schenone; M Grandis; C Gemelli; A Mauro; L G Pradotto; L Gentile; C Stancanelli; A Lozza; S Perlini; G Piscosquito; D Calabrese; A Mazzeo; L Obici; D Pareyson
Journal:  J Neurol       Date:  2016-03-16       Impact factor: 4.849

6.  Predictive model of response to tafamidis in hereditary ATTR polyneuropathy.

Authors:  Cecília Monteiro; Jaleh S Mesgazardeh; João Anselmo; Joana Fernandes; Marta Novais; Carla Rodrigues; Gabriel J Brighty; David L Powers; Evan T Powers; Teresa Coelho; Jeffery W Kelly
Journal:  JCI Insight       Date:  2019-06-20

7.  Splenic regression of amyloid on multi-modality imaging in response to treatment with patisiran and diflunisal in hereditary transthyretin amyloidosis.

Authors:  Rishi K Patel; Marianna Fontana; Philip N Hawkins; Julian Gillmore
Journal:  Amyloid       Date:  2021-05-04       Impact factor: 7.141

8.  Inhibition of the mechano-enzymatic amyloidogenesis of transthyretin: role of ligand affinity, binding cooperativity and occupancy of the inner channel.

Authors:  Guglielmo Verona; P Patrizia Mangione; Sara Raimondi; Sofia Giorgetti; Giulia Faravelli; Riccardo Porcari; Alessandra Corazza; Julian D Gillmore; Philip N Hawkins; Mark B Pepys; Graham W Taylor; Vittorio Bellotti
Journal:  Sci Rep       Date:  2017-03-15       Impact factor: 4.379

9.  Patient-reported burden of hereditary transthyretin amyloidosis on functioning and well-being.

Authors:  Andrew Lovley; Kimberly Raymond; Spencer D Guthrie; Michael Pollock; Vaishali Sanchorawala; Michelle K White
Journal:  J Patient Rep Outcomes       Date:  2021-01-07

10.  Estimating the global prevalence of transthyretin familial amyloid polyneuropathy.

Authors:  Hartmut H Schmidt; Márcia Waddington-Cruz; Marc F Botteman; John A Carter; Avijeet S Chopra; Markay Hopps; Michelle Stewart; Shari Fallet; Leslie Amass
Journal:  Muscle Nerve       Date:  2018-02-01       Impact factor: 3.217

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  2 in total

Review 1.  Genetic pain loss disorders.

Authors:  Annette Lischka; Petra Lassuthova; Arman Çakar; Christopher J Record; Jonas Van Lent; Jonathan Baets; Maike F Dohrn; Jan Senderek; Angelika Lampert; David L Bennett; John N Wood; Vincent Timmerman; Thorsten Hornemann; Michaela Auer-Grumbach; Yesim Parman; Christian A Hübner; Miriam Elbracht; Katja Eggermann; C Geoffrey Woods; James J Cox; Mary M Reilly; Ingo Kurth
Journal:  Nat Rev Dis Primers       Date:  2022-06-16       Impact factor: 65.038

2.  Peripheral Neuropathy: No Longer the Land of Therapeutic Nihilism.

Authors:  Michael Polydefkis; Charlotte J Sumner
Journal:  Neurotherapeutics       Date:  2022-01-06       Impact factor: 6.088

  2 in total

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