Electroretinogram abnormalities in FKRP-related limb–girdle muscular dystrophy (LGMDR9)

Hagedorn, Joshua; Dunn, Taylor M.; Bhattarai, Sajag; Stephan, Carrie M.; Mathews, Katherine D.; Pfeifer, Wanda; Drack, Arlene V.

doi:10.1007/s10633-022-09909-4

Cited by 1 publication

(1 citation statement)

References 28 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Some forms of congenital myopathy result in eye muscle weakness and ophthalmoparesis and are particularly amenable to study via the optokinetic response ( Jungbluth et al, 2018 ). Dystroglycanopathies and, to a lesser extent, DMD are linked to retinal deficits due to the critical role of the dystrophin-glycoprotein complex in photoreceptor synapse function ( Barboni et al, 2022 ; Hagedorn et al, 2023 ; Jahncke and Wright, 2023 ). These have not been extensively studied in the zebrafish, but lighting conditions and shifts in light-dark cycles could affect experimental outcomes as zebrafish have defined circadian rhythms ( Wolter and Svoboda, 2020 ; Pintos et al, 2023 ).…”

Section: Standards For Phenotypic Endpoints and Outcome Measures For ...mentioning

confidence: 99%

Standardization of zebrafish drug testing parameters for muscle diseases

Karuppasamy,

English,

Henry

et al. 2024

Disease Models &Amp; Mechanisms

View full text Add to dashboard Cite

Skeletal muscular diseases predominantly affect skeletal and cardiac muscle, resulting in muscle weakness, impaired respiratory function and decreased lifespan. These harmful outcomes lead to poor health-related quality of life and carry a high healthcare economic burden. The absence of promising treatments and new therapies for muscular disorders requires new methods for candidate drug identification and advancement in animal models. Consequently, the rapid screening of drug compounds in an animal model that mimics features of human muscle disease is warranted. Zebrafish are a versatile model in preclinical studies that support developmental biology and drug discovery programs for novel chemical entities and repurposing of established drugs. Due to several advantages, there is an increasing number of applications of the zebrafish model for high-throughput drug screening for human disorders and developmental studies. Consequently, standardization of key drug screening parameters, such as animal husbandry protocols, drug compound administration and outcome measures, is paramount for the continued advancement of the model and field. Here, we seek to summarize and explore critical drug treatment and drug screening parameters in the zebrafish-based modeling of human muscle diseases. Through improved standardization and harmonization of drug screening parameters and protocols, we aim to promote more effective drug discovery programs.

show abstract