As a Model System for Duchenne Muscular Dystrophy

Overview

Journal Int J Mol Sci

Publisher MDPI

Specialties Biochemistry
Chemistry
Molecular Biology

Date 2021 Jun 2

PMID 34063069

Citations 7

Authors

Rebecca A Ellwood

Mathew Piasecki

Nathaniel J Szewczyk

Affiliations

Soon will be listed here.

Abstract

The nematode worm has been used extensively to enhance our understanding of the human neuromuscular disorder Duchenne Muscular Dystrophy (DMD). With new arising clinically relevant models, technologies and treatments, there is a need to reconcile the literature and collate the key findings associated with this model.

Citing Articles

Aligning with the 3Rs: alternative models for research into muscle development and inherited myopathies.

Mehmood H, Kasher P, Barrett-Jolley R, Walmsley G BMC Vet Res. 2024; 20(1):477.

PMID: 39425123 PMC: 11488271. DOI: 10.1186/s12917-024-04309-z.

Characterization of muscle growth and sarcomere branching in the striated musculature of .

Fazyl A, Anbu A, Kollbaum S, Conklin E, Schroeder N, Vidal-Gadea A bioRxiv. 2024; .

PMID: 39257798 PMC: 11383985. DOI: 10.1101/2024.08.30.610496.

Cross-species modeling of muscular dystrophy in Caenorhabditis elegans using patient-derived extracellular vesicles.

Shalash R, Levi-Ferber M, Cohen C, Dori A, Brodie C, Henis-Korenblit S Dis Model Mech. 2024; 17(3).

PMID: 38501170 PMC: 11007864. DOI: 10.1242/dmm.050412.

Increased mitochondrial Ca contributes to health decline with age and Duchene muscular dystrophy in C. elegans.

Higashitani A, Teranishi M, Nakagawa Y, Itoh Y, Sudevan S, Szewczyk N FASEB J. 2023; 37(4):e22851.

PMID: 36935171 PMC: 10946577. DOI: 10.1096/fj.202201489RR.

Sulfur amino acid supplementation displays therapeutic potential in a C. elegans model of Duchenne muscular dystrophy.

Ellwood R, Slade L, Lewis J, Torregrossa R, Sudevan S, Piasecki M Commun Biol. 2022; 5(1):1255.

PMID: 36385509 PMC: 9668843. DOI: 10.1038/s42003-022-04212-z.

References

De Luca A, Nico B, Liantonio A, Didonna M, Fraysse B, Pierno S . A multidisciplinary evaluation of the effectiveness of cyclosporine a in dystrophic mdx mice. Am J Pathol. 2005; 166(2):477-89. PMC: 1602333. DOI: 10.1016/S0002-9440(10)62270-5. View

Culetto E, Sattelle D . A role for Caenorhabditis elegans in understanding the function and interactions of human disease genes. Hum Mol Genet. 2000; 9(6):869-77. DOI: 10.1093/hmg/9.6.869. View

Murphy D, Mendell J, Engel W . Serotonin and platelet function in Duchenne muscular dystrophy. Arch Neurol. 1973; 28(4):239-42. DOI: 10.1001/archneur.1973.00490220047006. View

Karpati G, Carpenter S, Prescott S . Small-caliber skeletal muscle fibers do not suffer necrosis in mdx mouse dystrophy. Muscle Nerve. 1988; 11(8):795-803. DOI: 10.1002/mus.880110802. View

Sznitman J, Purohit P, Krajacic P, Lamitina T, Arratia P . Material properties of Caenorhabditis elegans swimming at low Reynolds number. Biophys J. 2010; 98(4):617-26. PMC: 2820645. DOI: 10.1016/j.bpj.2009.11.010. View

Schiavone M, Zulian A, Menazza S, Petronilli V, Argenton F, Merlini L . Alisporivir rescues defective mitochondrial respiration in Duchenne muscular dystrophy. Pharmacol Res. 2017; 125(Pt B):122-131. DOI: 10.1016/j.phrs.2017.09.001. View

Brouilly N, Lecroisey C, Martin E, Pierson L, Mariol M, Qadota H . Ultra-structural time-course study in the C. elegans model for Duchenne muscular dystrophy highlights a crucial role for sarcomere-anchoring structures and sarcolemma integrity in the earliest steps of the muscle degeneration process. Hum Mol Genet. 2015; 24(22):6428-45. DOI: 10.1093/hmg/ddv353. View

Chahbouni M, Escames G, Venegas C, Sevilla B, Garcia J, Lopez L . Melatonin treatment normalizes plasma pro-inflammatory cytokines and nitrosative/oxidative stress in patients suffering from Duchenne muscular dystrophy. J Pineal Res. 2010; 48(3):282-289. DOI: 10.1111/j.1600-079X.2010.00752.x. View

Kim H, Pierce-Shimomura J, Oh H, Johnson B, Goodman M, McIntire S . The dystrophin complex controls bk channel localization and muscle activity in Caenorhabditis elegans. PLoS Genet. 2009; 5(12):e1000780. PMC: 2788698. DOI: 10.1371/journal.pgen.1000780. View

10.

Beron C, Vidal-Gadea A, Cohn J, Parikh A, Hwang G, Pierce-Shimomura J . The burrowing behavior of the nematode Caenorhabditis elegans: a new assay for the study of neuromuscular disorders. Genes Brain Behav. 2015; 14(4):357-68. PMC: 4444045. DOI: 10.1111/gbb.12217. View

11.

Blake D, Weir A, Newey S, Davies K . Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol Rev. 2002; 82(2):291-329. DOI: 10.1152/physrev.00028.2001. View

12.

Manning J, Kulbida R, Rai P, Jensen L, Bouma J, Singh S . Amitriptyline is efficacious in ameliorating muscle inflammation and depressive symptoms in the mdx mouse model of Duchenne muscular dystrophy. Exp Physiol. 2014; 99(10):1370-86. DOI: 10.1113/expphysiol.2014.079475. View

13.

Briguet A, Erb M, Courdier-Fruh I, Barzaghi P, Santos G, Herzner H . Effect of calpain and proteasome inhibition on Ca2+-dependent proteolysis and muscle histopathology in the mdx mouse. FASEB J. 2008; 22(12):4190-200. DOI: 10.1096/fj.07-099036. View

14.

Carre-Pierrat M, Grisoni K, Gieseler K, Mariol M, Martin E, Jospin M . The SLO-1 BK channel of Caenorhabditis elegans is critical for muscle function and is involved in dystrophin-dependent muscle dystrophy. J Mol Biol. 2006; 358(2):387-95. DOI: 10.1016/j.jmb.2006.02.037. View

15.

Belosludtsev K, Dubinin M, Belosludtseva N, Mironova G . Mitochondrial Ca2+ Transport: Mechanisms, Molecular Structures, and Role in Cells. Biochemistry (Mosc). 2019; 84(6):593-607. DOI: 10.1134/S0006297919060026. View

16.

Lee A, Perreault R, Harel S, Boulier E, Suderman M, Hallett M . Searching for signaling balance through the identification of genetic interactors of the Rab guanine-nucleotide dissociation inhibitor gdi-1. PLoS One. 2010; 5(5):e10624. PMC: 2869356. DOI: 10.1371/journal.pone.0010624. View

17.

Chahbouni M, Escames G, Lopez L, Sevilla B, Doerrier C, Munoz-Hoyos A . Melatonin treatment counteracts the hyperoxidative status in erythrocytes of patients suffering from Duchenne muscular dystrophy. Clin Biochem. 2011; 44(10-11):853-8. DOI: 10.1016/j.clinbiochem.2011.04.001. View

18.

Hollinger K, Selsby J . The physiological response of protease inhibition in dystrophic muscle. Acta Physiol (Oxf). 2013; 208(3):234-44. DOI: 10.1111/apha.12114. View

19.

OReilly L, Luke C, Perlmutter D, Silverman G, Pak S . C. elegans in high-throughput drug discovery. Adv Drug Deliv Rev. 2013; 69-70:247-53. PMC: 4019719. DOI: 10.1016/j.addr.2013.12.001. View

20.

Valladares D, Utreras-Mendoza Y, Campos C, Morales C, Diaz-Vegas A, Contreras-Ferrat A . IP receptor blockade restores autophagy and mitochondrial function in skeletal muscle fibers of dystrophic mice. Biochim Biophys Acta Mol Basis Dis. 2018; 1864(11):3685-3695. DOI: 10.1016/j.bbadis.2018.08.042. View