Extracellular Vesicles from Regenerating Skeletal Muscle Mitigate Muscle Atrophy in an Amyotrophic Lateral Sclerosis Mouse Model

Amyotrophic lateral sclerosis (ALS) is a devastating neuromuscular disease characterized by progressive motor neuron degeneration and muscle atrophy, with no effective treatments available. Chronic inflammation, which impairs muscle regeneration and promotes proteolysis, is a key contributor to ALS-...

Full description

Saved in:
Bibliographic Details
Main Authors: Jinghui Gao, Aria Sikal, Rachel Hankin, Yaochao Zheng, Elijah Sterling, Kenny Chan, Yao Yao
Format: Article
Language:English
Published: MDPI AG 2025-03-01
Series:Cells
Subjects:
Online Access:https://www.mdpi.com/2073-4409/14/6/464
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849342767568781312
author Jinghui Gao
Aria Sikal
Rachel Hankin
Yaochao Zheng
Elijah Sterling
Kenny Chan
Yao Yao
author_facet Jinghui Gao
Aria Sikal
Rachel Hankin
Yaochao Zheng
Elijah Sterling
Kenny Chan
Yao Yao
author_sort Jinghui Gao
collection DOAJ
description Amyotrophic lateral sclerosis (ALS) is a devastating neuromuscular disease characterized by progressive motor neuron degeneration and muscle atrophy, with no effective treatments available. Chronic inflammation, which impairs muscle regeneration and promotes proteolysis, is a key contributor to ALS-related muscle atrophy and a promising therapeutic target. Here, we applied extracellular vesicles (EVs) derived from regenerating skeletal muscles 14 days post-acute injury (CTXD14SkM-EVs), which possess a unique anti-inflammatory profile, to target muscle defects in ALS. We found that CTXD14SkM-EVs enhanced myoblast differentiation and fusion in a cellular muscle-wasting model induced by pro-inflammatory cytokine tumor necrosis factor alpha. Intramuscular administration of these EVs into an ALS mouse model mitigated muscle atrophy by promoting muscle regeneration, shifting macrophage polarization from pro-inflammatory M1 to anti-inflammatory M2 state, and suppressing the aberrant Nuclear Factor Kappa B (NF-κB) signaling, a key driver of muscle protein degradation. These results underscore the therapeutic potential of regenerating muscle-derived EVs for combating muscle atrophy in ALS.
format Article
id doaj-art-a960a830a4994fcaa4dcad179e494830
institution Kabale University
issn 2073-4409
language English
publishDate 2025-03-01
publisher MDPI AG
record_format Article
series Cells
spelling doaj-art-a960a830a4994fcaa4dcad179e4948302025-08-20T03:43:15ZengMDPI AGCells2073-44092025-03-0114646410.3390/cells14060464Extracellular Vesicles from Regenerating Skeletal Muscle Mitigate Muscle Atrophy in an Amyotrophic Lateral Sclerosis Mouse ModelJinghui Gao0Aria Sikal1Rachel Hankin2Yaochao Zheng3Elijah Sterling4Kenny Chan5Yao Yao6Regenerative Bioscience Center, Department of Animal and Dairy Science, College of Agricultural and Environmental Science, University of Georgia, Athens, GA 30602, USARegenerative Bioscience Center, Department of Animal and Dairy Science, College of Agricultural and Environmental Science, University of Georgia, Athens, GA 30602, USARegenerative Bioscience Center, Department of Animal and Dairy Science, College of Agricultural and Environmental Science, University of Georgia, Athens, GA 30602, USARegenerative Bioscience Center, Department of Animal and Dairy Science, College of Agricultural and Environmental Science, University of Georgia, Athens, GA 30602, USARegenerative Bioscience Center, Department of Animal and Dairy Science, College of Agricultural and Environmental Science, University of Georgia, Athens, GA 30602, USADepartment of Physiology and Pharmacology, College of Veterinary Medicine, University of Georgia, Athens, GA 30602, USARegenerative Bioscience Center, Department of Animal and Dairy Science, College of Agricultural and Environmental Science, University of Georgia, Athens, GA 30602, USAAmyotrophic lateral sclerosis (ALS) is a devastating neuromuscular disease characterized by progressive motor neuron degeneration and muscle atrophy, with no effective treatments available. Chronic inflammation, which impairs muscle regeneration and promotes proteolysis, is a key contributor to ALS-related muscle atrophy and a promising therapeutic target. Here, we applied extracellular vesicles (EVs) derived from regenerating skeletal muscles 14 days post-acute injury (CTXD14SkM-EVs), which possess a unique anti-inflammatory profile, to target muscle defects in ALS. We found that CTXD14SkM-EVs enhanced myoblast differentiation and fusion in a cellular muscle-wasting model induced by pro-inflammatory cytokine tumor necrosis factor alpha. Intramuscular administration of these EVs into an ALS mouse model mitigated muscle atrophy by promoting muscle regeneration, shifting macrophage polarization from pro-inflammatory M1 to anti-inflammatory M2 state, and suppressing the aberrant Nuclear Factor Kappa B (NF-κB) signaling, a key driver of muscle protein degradation. These results underscore the therapeutic potential of regenerating muscle-derived EVs for combating muscle atrophy in ALS.https://www.mdpi.com/2073-4409/14/6/464amyotrophic lateral sclerosisregenerating skeletal muscleextracellular vesiclesmuscle atrophy
spellingShingle Jinghui Gao
Aria Sikal
Rachel Hankin
Yaochao Zheng
Elijah Sterling
Kenny Chan
Yao Yao
Extracellular Vesicles from Regenerating Skeletal Muscle Mitigate Muscle Atrophy in an Amyotrophic Lateral Sclerosis Mouse Model
Cells
amyotrophic lateral sclerosis
regenerating skeletal muscle
extracellular vesicles
muscle atrophy
title Extracellular Vesicles from Regenerating Skeletal Muscle Mitigate Muscle Atrophy in an Amyotrophic Lateral Sclerosis Mouse Model
title_full Extracellular Vesicles from Regenerating Skeletal Muscle Mitigate Muscle Atrophy in an Amyotrophic Lateral Sclerosis Mouse Model
title_fullStr Extracellular Vesicles from Regenerating Skeletal Muscle Mitigate Muscle Atrophy in an Amyotrophic Lateral Sclerosis Mouse Model
title_full_unstemmed Extracellular Vesicles from Regenerating Skeletal Muscle Mitigate Muscle Atrophy in an Amyotrophic Lateral Sclerosis Mouse Model
title_short Extracellular Vesicles from Regenerating Skeletal Muscle Mitigate Muscle Atrophy in an Amyotrophic Lateral Sclerosis Mouse Model
title_sort extracellular vesicles from regenerating skeletal muscle mitigate muscle atrophy in an amyotrophic lateral sclerosis mouse model
topic amyotrophic lateral sclerosis
regenerating skeletal muscle
extracellular vesicles
muscle atrophy
url https://www.mdpi.com/2073-4409/14/6/464
work_keys_str_mv AT jinghuigao extracellularvesiclesfromregeneratingskeletalmusclemitigatemuscleatrophyinanamyotrophiclateralsclerosismousemodel
AT ariasikal extracellularvesiclesfromregeneratingskeletalmusclemitigatemuscleatrophyinanamyotrophiclateralsclerosismousemodel
AT rachelhankin extracellularvesiclesfromregeneratingskeletalmusclemitigatemuscleatrophyinanamyotrophiclateralsclerosismousemodel
AT yaochaozheng extracellularvesiclesfromregeneratingskeletalmusclemitigatemuscleatrophyinanamyotrophiclateralsclerosismousemodel
AT elijahsterling extracellularvesiclesfromregeneratingskeletalmusclemitigatemuscleatrophyinanamyotrophiclateralsclerosismousemodel
AT kennychan extracellularvesiclesfromregeneratingskeletalmusclemitigatemuscleatrophyinanamyotrophiclateralsclerosismousemodel
AT yaoyao extracellularvesiclesfromregeneratingskeletalmusclemitigatemuscleatrophyinanamyotrophiclateralsclerosismousemodel