Literature DB >> 32917814

Disruption of a key ligand-H-bond network drives dissociative properties in vamorolone for Duchenne muscular dystrophy treatment.

Xu Liu1, Yashuo Wang1, Jennifer S Gutierrez1, Jesse M Damsker2, Kanneboyina Nagaraju2,3, Eric P Hoffman2,3, Eric A Ortlund4.   

Abstract

Duchenne muscular dystrophy is a genetic disorder that shows chronic and progressive damage to skeletal and cardiac muscle leading to premature death. Antiinflammatory corticosteroids targeting the glucocorticoid receptor (GR) are the current standard of care but drive adverse side effects such as deleterious bone loss. Through subtle modification to a steroidal backbone, a recently developed drug, vamorolone, appears to preserve beneficial efficacy but with significantly reduced side effects. We use combined structural, biophysical, and biochemical approaches to show that loss of a receptor-ligand hydrogen bond drives these remarkable therapeutic effects. Moreover, vamorolone uniformly weakens coactivator associations but not corepressor associations, implicating partial agonism as the main driver of its dissociative properties. Additionally, we identify a critical and evolutionarily conserved intramolecular network connecting the ligand to the coregulator binding surface. Interruption of this allosteric network by vamorolone selectively reduces GR-driven transactivation while leaving transrepression intact. Our results establish a mechanistic understanding of how vamorolone reduces side effects, guiding the future design of partial agonists as selective GR modulators with an improved therapeutic index.

Entities:  

Keywords:  allostery; dissociative agonist; glucocorticoid receptor; molecular dynamics; nuclear receptor

Mesh:

Substances:

Year:  2020        PMID: 32917814      PMCID: PMC7533876          DOI: 10.1073/pnas.2006890117

Source DB:  PubMed          Journal:  Proc Natl Acad Sci U S A        ISSN: 0027-8424            Impact factor:   11.205


  59 in total

1.  The three-dimensional structures of antagonistic and agonistic forms of the glucocorticoid receptor ligand-binding domain: RU-486 induces a transconformation that leads to active antagonism.

Authors:  Björn Kauppi; Clarissa Jakob; Mathias Färnegårdh; Jie Yang; Harri Ahola; Maria Alarcon; Karin Calles; Owe Engström; John Harlan; Steven Muchmore; Anna-Karin Ramqvist; Susanne Thorell; Lars Ohman; Jonathan Greer; Jan-Ake Gustafsson; Jan Carlstedt-Duke; Mats Carlquist
Journal:  J Biol Chem       Date:  2003-04-09       Impact factor: 5.157

2.  A ligand-mediated hydrogen bond network required for the activation of the mineralocorticoid receptor.

Authors:  Randy K Bledsoe; Kevin P Madauss; Jason A Holt; Christopher J Apolito; Millard H Lambert; Kenneth H Pearce; Thomas B Stanley; Eugene L Stewart; Ryan P Trump; Timothy M Willson; Shawn P Williams
Journal:  J Biol Chem       Date:  2005-06-20       Impact factor: 5.157

3.  Structures and mechanism for the design of highly potent glucocorticoids.

Authors:  Yuanzheng He; Wei Yi; Kelly Suino-Powell; X Edward Zhou; W David Tolbert; Xiaobo Tang; Jing Yang; Huaiyu Yang; Jingjing Shi; Li Hou; Hualiang Jiang; Karsten Melcher; H Eric Xu
Journal:  Cell Res       Date:  2014-04-25       Impact factor: 25.617

4.  Linking ligand-induced alterations in androgen receptor structure to differential gene expression: a first step in the rational design of selective androgen receptor modulators.

Authors:  Dmitri Kazmin; Tatiana Prytkova; C Edgar Cook; Russell Wolfinger; Tzu-Ming Chu; David Beratan; J D Norris; Ching-yi Chang; Donald P McDonnell
Journal:  Mol Endocrinol       Date:  2006-03-30

5.  Evolution of steroid receptors from an estrogen-sensitive ancestral receptor.

Authors:  Geeta N Eick; Joseph W Thornton
Journal:  Mol Cell Endocrinol       Date:  2010-09-15       Impact factor: 4.102

6.  The antagonists but not partial agonists of glucocorticoid receptor ligands show substantial side effect dissociation.

Authors:  Xiao Hu; Sarah Du; Cansu Tunca; Timothy Braden; Kelly R Long; Jamie Lee; Elizabeth G Webb; Jessica D Dietz; Susan Hummert; Sharon Rouw; Shridhar G Hegde; R Keith Webber; Mark G Obukowicz
Journal:  Endocrinology       Date:  2011-05-10       Impact factor: 4.736

Review 7.  Glucocorticoid receptor control of transcription: precision and plasticity via allostery.

Authors:  Emily R Weikum; Matthew T Knuesel; Eric A Ortlund; Keith R Yamamoto
Journal:  Nat Rev Mol Cell Biol       Date:  2017-01-05       Impact factor: 94.444

8.  Engineered luciferase reporter from a deep sea shrimp utilizing a novel imidazopyrazinone substrate.

Authors:  Mary P Hall; James Unch; Brock F Binkowski; Michael P Valley; Braeden L Butler; Monika G Wood; Paul Otto; Kristopher Zimmerman; Gediminas Vidugiris; Thomas Machleidt; Matthew B Robers; Hélène A Benink; Christopher T Eggers; Michael R Slater; Poncho L Meisenheimer; Dieter H Klaubert; Frank Fan; Lance P Encell; Keith V Wood
Journal:  ACS Chem Biol       Date:  2012-08-30       Impact factor: 5.100

9.  Vamorolone targets dual nuclear receptors to treat inflammation and dystrophic cardiomyopathy.

Authors:  Christopher R Heier; Qing Yu; Alyson A Fiorillo; Christopher B Tully; Asya Tucker; Davi A Mazala; Kitipong Uaesoontrachoon; Sadish Srinivassane; Jesse M Damsker; Eric P Hoffman; Kanneboyina Nagaraju; Christopher F Spurney
Journal:  Life Sci Alliance       Date:  2019-02-11

Review 10.  Deflazacort for the treatment of Duchenne Dystrophy: a systematic review.

Authors:  Craig Campbell; Pierre Jacob
Journal:  BMC Neurol       Date:  2003-09-08       Impact factor: 2.474

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  7 in total

1.  Stereoisomers of an Aryl Pyrazole Glucocorticoid Receptor Agonist Scaffold Elicit Differing Anti-inflammatory Responses.

Authors:  Ashley M Lato; Susan J Burke; Maggie P Ducote; Brandon J Kennedy; J Jason Collier; Shawn R Campagna
Journal:  ACS Med Chem Lett       Date:  2022-08-22       Impact factor: 4.632

Review 2.  Structural insights into glucocorticoid receptor function.

Authors:  Filipp Frank; Xu Liu; Eric A Ortlund
Journal:  Biochem Soc Trans       Date:  2021-11-01       Impact factor: 4.919

3.  Genome-wide binding potential and regulatory activity of the glucocorticoid receptor's monomeric and dimeric forms.

Authors:  Thomas A Johnson; Ville Paakinaho; Sohyoung Kim; Gordon L Hager; Diego M Presman
Journal:  Nat Commun       Date:  2021-03-31       Impact factor: 14.919

4.  Efficacy and Safety of Vamorolone in Duchenne Muscular Dystrophy: A 30-Month Nonrandomized Controlled Open-Label Extension Trial.

Authors:  Jean K Mah; Paula R Clemens; Michela Guglieri; Edward C Smith; Richard S Finkel; Mar Tulinius; Yoram Nevo; Monique M Ryan; Richard Webster; Diana Castro; Nancy L Kuntz; Craig M McDonald; Jesse M Damsker; Benjamin D Schwartz; Laurel J Mengle-Gaw; Stefan Jackowski; Georgia Stimpson; Deborah A Ridout; Vandana Ayyar-Gupta; Giovanni Baranello; Adnan Y Manzur; Francesco Muntoni; Heather Gordish-Dressman; Mika Leinonen; Leanne M Ward; Eric P Hoffman; Utkarsh J Dang
Journal:  JAMA Netw Open       Date:  2022-01-04

5.  Muscle mitochondrial remodeling by intermittent glucocorticoid drugs requires an intact circadian clock and muscle PGC1α.

Authors:  Mattia Quattrocelli; Michelle Wintzinger; Karen Miz; Daniel C Levine; Clara Bien Peek; Joseph Bass; Elizabeth M McNally
Journal:  Sci Adv       Date:  2022-02-18       Impact factor: 14.136

6.  Glucocorticoid receptor condensates link DNA-dependent receptor dimerization and transcriptional transactivation.

Authors:  Filipp Frank; Xu Liu; Eric A Ortlund
Journal:  Proc Natl Acad Sci U S A       Date:  2021-07-27       Impact factor: 11.205

7.  New Insight Into the Structure-Activity Relationship of Sweet-Tasting Proteins: Protein Sector and Its Role for Sweet Properties.

Authors:  Xiangzhong Zhao; Congrui Wang; Yue Zheng; Bo Liu
Journal:  Front Nutr       Date:  2021-06-18
  7 in total

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