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Fhl2b expression ameliorates muscular dystrophy [5dpf trunk]

ABSTRACT: In muscle dystrophies, muscle fibers loose integrity and die, leading to significant suffering and a shorter life. Strikingly, the extraocular muscles (EOMs), controlling eye movements, are spared and function well despite the disease progression. Although EOMs have been shown to have important differences compared to body musculature the mechanisms underlying this inherent resistance to muscle dystrophies remain largely unknown. Here, we demonstrate important differences in gene expression as a response to muscle dystrophies between the EOMs and trunk muscle in zebrafish via transcriptomic profiling. We show that the LIM-protein Fhl2 is upregulated in response to knockout of desmin, plectin and obscurin, intermediate filament proteins causing different muscle dystrophies, and contributes to disease protection of the EOMs. Moreover, we show that ectopic expression of fhl2b can partially rescue the muscle phenotype in the zebrafish Duchenne muscular dystrophy model sapje, significantly improving their survival rate. Therefore, fhl2 is a protective agent and a candidate target gene for therapy of muscle dystrophies.

ORGANISM(S): Danio rerio

PROVIDER: GSE242135 | GEO | 2024/02/08

REPOSITORIES: GEO

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fhl2b expression ameliorates muscular dystrophy [5 months EOM and trunk]

Project description:In muscle dystrophies, muscle fibers loose integrity and die, leading to significant suffering and a shorter life. Strikingly, the extraocular muscles (EOMs), controlling eye movements, are spared and function well despite the disease progression. Although EOMs have been shown to have important differences compared to body musculature the mechanisms underlying this inherent resistance to muscle dystrophies remain largely unknown. Here, we demonstrate important differences in gene expression as a response to muscle dystrophies between the EOMs and trunk muscle in zebrafish via transcriptomic profiling. We show that the LIM-protein Fhl2 is upregulated in response to knockout of desmin, plectin and obscurin, intermediate filament proteins causing different muscle dystrophies, and contributes to disease protection of the EOMs. Moreover, we show that ectopic expression of fhl2b can partially rescue the muscle phenotype in the zebrafish Duchenne muscular dystrophy model sapje, significantly improving their survival rate. Therefore, fhl2 is a protective agent and a candidate target gene for therapy of muscle dystrophies.

2024-02-08 | GSE242136 | GEO

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Influence of hyperthyroid conditions on gene expression in rat extraocular muscles

Project description:Extraocular muscles (EOMs) are a highly specialized type of tissue with a wide range of unique properties, including characteristic innervation, development, and structural proteins. Even though EOMs are frequently and prominently involved in thyroid-associated diseases, little is known about the immediate effects of thyroid hormone on these muscles. In order to create a comprehensive profile of changes in gene expression levels in EOMs induced by thyroid hormone, hyperthyroid conditions were simulated by treating adult Sprague-Dawley rats with intraperitoneal injections of 25 μg T3 per 100 g body weight over the course of six weeks; subsequently, microarray analysis was used to determine changes in mRNA levels in EOMs from T3-treated animals relative to untreated controls. Adult Sprague-Dawley rats (initial body weight 200 ± 40 g) were fed ad libitum with standard laboratory diet and tap water. Eight rats were randomly divided into a T3-treated and a control group. To simulate a chronically hyperthyroid state, T3 treatment was administered by intraperitoneal injections of 25 µg T3 (Sigma-Aldrich) per 100 g body weight. Injections were administered every second day over a period of six weeks. 24 hours after the last injection, the animals were sacrificed by CO2 asphyxiation. For RNA isolation, the extraocular muscles (EOMs) were removed, flash-frozen in liquid nitrogen and stored at -80 °C until further use.

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Project description:Extraocular muscles (EOMs) are a highly specialized type of tissue with a wide range of unique properties, including characteristic innervation, development, and structural proteins. Even though EOMs are frequently and prominently involved in thyroid-associated diseases, little is known about the immediate effects of thyroid hormone on these muscles. In order to create a comprehensive profile of changes in gene expression levels in EOMs induced by thyroid hormone, hyperthyroid conditions were simulated by treating adult Sprague-Dawley rats with intraperitoneal injections of 25 ?g T3 per 100 g body weight over the course of six weeks; subsequently, microarray analysis was used to determine changes in mRNA levels in EOMs from T3-treated animals relative to untreated controls. RNA isolated from tibialis anterior muscles of the same animals was used as a reference fast-twitch muscle. Adult Sprague-Dawley rats (initial body weight 200 ± 40 g) were fed ad libitum with standard laboratory diet and tap water. Eight rats were randomly divided into a T3-treated and a control group, three males and one female each. To simulate a chronically hyperthyroid state, T3 treatment was administered by intraperitoneal injections of 25 µg T3 (Sigma-Aldrich, St. Louis, MO, U.S.A.) per 100 g body weight. Injections were administered every second day over a period of six weeks. 24 hours after the last injection, the animals were sacrificed by CO2 asphyxiation, muscles were dissected immediately after death. Each of the four biological replicates in each group was used for a single microarray each. RNA from EOMs was used. Tibialis anterior RNA from the same animals served as a fast-twitch reference muscle.

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Project description:The fast and constant activity of the extraocular muscles (EOMs) impose mechanical and metabolic stresses not typically seen in limb skeletal muscles. These functional properties may explain why EOMs seem to age at a faster rate than other skeletal muscles. Using high-density cDNA microarrays, this study investigated the gene expression profile of EOMs and extensor digitorum longus muscles (EDL) of Fischer 344/Brown Norway F1 hybrid rats at 6-, 18- and 30-months of age. At 6-mo, 705 genes and expressed sequence tags (ESTs) were differentially expressed in EOMs (436 up, 269 down). Overall, the EOM profile at this age was mostly consistent with the increased expression of fetal, developmental and EOM-specific myosin isoforms, enzymes involved in glycolysis and TCA cycle, and ion transporters and pumps, confirming the notion that EOM may represent a distinct muscle group (PNAS 98:12062, 2001). Interestingly, at 18-mo only 36 probes were significantly different in EOM (15 up, 21 down), most of them ESTs. However, at 30-mo EOMs had 655 differentially expressed genes and ESTs (480 up, 175 down). In this age group, the EOM expression profile reverted to a pattern similar to that found at 6-mo, with evidence of ongoing tissue remodeling and increased expression of antioxidant enzymes. These results indicate that the gene expression profile of EOM and EDL evolve differently throughout the lifespan. Keywords: aging, muscle type comparison

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