Literature DB >> 31302339

First clinical trials of novel ENaC targeting therapy, SPX-101, in healthy volunteers and adults with cystic fibrosis.

Peter Couroux1, Paulina Farias2, Leena Rizvi3, Katherine Griffin3, Christie Hudson2, Timothy Crowder4, Robert Tarran5, Elizabeth Tullis6.   

Abstract

BACKGROUND: ENaC inhibition has been investigated as a CF treatment; however, small molecule inhibitors of ENaC lack efficacy and/or have shown dose-limiting hyperkalemia. SPX-101 is a novel, investigational small peptide (SPLUNC1 mimetic) that regulates ENaC density with the potential for efficacy without systemic effects.
METHODS: Two trials are presented: The first was a Phase 1, 2-part, randomized, double-blind, placebo-controlled, ascending-dose study of nebulized SPX-101 in healthy adults. Part 1 evaluated 4 single doses of SPX-101 ranging from 20 to 240 mg. Part 2 evaluated a 14-day regimen of SPX-101 at 4 doses of SPX-101 ranging from 10 to 120 mg BID. Pharmacokinetics, adverse events, spirometry, vital signs, electrocardiograms, pulse oximetry, and clinical laboratory values were assessed. The second trial was a tolerability-confirming, Phase 1b, open-label study conducted in 5 adult subjects with CF. Ascending doses of SPX-101 inhalation solution (10 mg-120 mg BID) were administered for 7 days. Safety was assessed as described above.
RESULTS: All 64 healthy volunteers (32 in each Part) completed the single and multiple dose study. SPX-101 was well tolerated with little/no systemic exposure and with no hyperkalemia. Adverse events were generally mild with reported respiratory events associated with the purported pharmacological activity of SPX-101. Tolerability of SPX-101 was similarly observed in adults with CF; all 5 subjects treated with SPX-101 completed the study.
CONCLUSIONS: SPX-101 was well-tolerated across a range of doses and had little/no systemic exposure in healthy adults and adults with CF, thus supporting further study in patients with CF. CLINICALTRIAL. GOV REGISTRATION: NCT03056989.
Copyright © 2019. Published by Elsevier Ltd.

Entities:  

Keywords:  Cystic fibrosis; ENaC inhibitor; ENaC modulator; SPLUNC1; SPX-101

Year:  2019        PMID: 31302339     DOI: 10.1016/j.pupt.2019.101819

Source DB:  PubMed          Journal:  Pulm Pharmacol Ther        ISSN: 1094-5539            Impact factor:   3.410


  7 in total

1.  A SPLUNC1 Peptidomimetic Inhibits Orai1 and Reduces Inflammation in a Murine Allergic Asthma Model.

Authors:  Joe A Wrennall; Saira Ahmad; Erin N Worthington; Tongde Wu; Alexandra S Goriounova; Alexis S Voeller; Ian E Stewart; Arunava Ghosh; Krzysztof Krajewski; Steven L Tilley; Anthony J Hickey; M Flori Sassano; Robert Tarran
Journal:  Am J Respir Cell Mol Biol       Date:  2022-03       Impact factor: 6.914

2.  Engineered mutant α-ENaC subunit mRNA delivered by lipid nanoparticles reduces amiloride currents in cystic fibrosis-based cell and mice models.

Authors:  Anindit Mukherjee; Kelvin D MacDonald; Jeonghwan Kim; Michael I Henderson; Yulia Eygeris; Gaurav Sahay
Journal:  Sci Adv       Date:  2020-11-18       Impact factor: 14.136

3.  Preclinical evaluation of the epithelial sodium channel inhibitor BI 1265162 for treatment of cystic fibrosis.

Authors:  Peter Nickolaus; Birgit Jung; Juan Sabater; Samuel Constant; Abhya Gupta
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Review 4.  Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis.

Authors:  James A Reihill; Lisa E J Douglas; S Lorraine Martin
Journal:  Genes (Basel)       Date:  2021-03-22       Impact factor: 4.096

5.  Novel therapeutic approaches for the management of cystic fibrosis.

Authors:  Ryan Jaques; Arslan Shakeel; Cameron Hoyle
Journal:  Multidiscip Respir Med       Date:  2020-11-26

Review 6.  Pharmacological Modulation of Ion Channels for the Treatment of Cystic Fibrosis.

Authors:  Madalena C Pinto; Iris A L Silva; Miquéias Lopes-Pacheco; Miriam F Figueira; Margarida D Amaral
Journal:  J Exp Pharmacol       Date:  2021-07-23

7.  Efficacy and safety of inhaled ENaC inhibitor BI 1265162 in patients with cystic fibrosis: BALANCE-CF 1, a randomised, phase II study.

Authors:  Christopher H Goss; Isabelle Fajac; Raksha Jain; Wolfgang Seibold; Abhya Gupta; Ming-Chi Hsu; Sivagurunathan Sutharsan; Jane C Davies; Marcus A Mall
Journal:  Eur Respir J       Date:  2022-02-17       Impact factor: 16.671

  7 in total

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