CHAMP1 is an essential regulator for human myoblast fusion and muscle development.
Fuente:
PubMed
Enregistré dans:
| Auteurs principaux: | , , , , , , , , , , , , |
|---|---|
| Format: | Artículo científico |
| Langue: | en |
| Publié: |
Nature communications
2026
|
| Sujets: | |
| Accès en ligne: | |
| Tags: |
Ajouter un tag
Pas de tags, Soyez le premier à ajouter un tag!
|
| _version_ | 1868266100197687297 |
|---|---|
| author | Zhang, Haifeng Zhou, Min Zhang, Zheng Wang, Zhaoning Shi, Ruifeng Wang, Yushu Wei, Xiaolin Shang, Renjie Li, Jianwen He, Cuiyu Xie, Jin Diao, Yarui Bi, Pengpeng |
| author_facet | Zhang, Haifeng Zhou, Min Zhang, Zheng Wang, Zhaoning Shi, Ruifeng Wang, Yushu Wei, Xiaolin Shang, Renjie Li, Jianwen He, Cuiyu Xie, Jin Diao, Yarui Bi, Pengpeng Zhang, Haifeng Zhou, Min Zhang, Zheng Wang, Zhaoning Shi, Ruifeng Wang, Yushu Wei, Xiaolin Shang, Renjie Li, Jianwen He, Cuiyu Xie, Jin Diao, Yarui Bi, Pengpeng |
| collection | PubMed - marine biology |
| contents | CHAMP1 is an essential regulator for human myoblast fusion and muscle development. Zhang, Haifeng Zhou, Min Zhang, Zheng Wang, Zhaoning Shi, Ruifeng Wang, Yushu Wei, Xiaolin Shang, Renjie Li, Jianwen He, Cuiyu Xie, Jin Diao, Yarui Bi, Pengpeng Humans Muscle Development Myoblasts MyoD Protein Cell Fusion Muscle, Skeletal Animals Phosphoproteins Mutation Human skeletal muscle comprises myofibers formed by fusion of thousands of myoblasts. This process depends on tightly regulated, muscle-specific fusogens, but its genetic control remains poorly understood. Here, we identify CHAMP1 (Chromosome Alignment Maintaining Phosphoprotein 1) as essential for human myoblast fusion in vitro and in vivo. Genomic and protein-interaction assays reveal a noncanonical role for CHAMP1 as a MyoD cofactor that directly activates expression of the key muscle fusogen Myomaker. As established in prior clinical reports, CHAMP1 mutations in patients cause developmental delay, hypotonia, and muscle weakness. Consistently, patient-derived cells show fusion defects that can be fully rescued by restoring Myomaker expression. Structure and function analyses identify C2H2-type zinc-finger motifs on CHAMP1 protein that are both necessary and sufficient for MyoD interaction and Myomaker expression. These findings highlight a cell-autonomous role for CHAMP1 in muscle development and disease and point to therapeutic avenues for treating CHAMP1-related muscle development defects. |
| format | Artículo científico |
| id | pubmed_41540007 |
| institution | PubMed |
| language | en |
| publishDate | 2026 |
| publisher | Nature communications |
| record_format | pubmed |
| spellingShingle | CHAMP1 is an essential regulator for human myoblast fusion and muscle development. Zhang, Haifeng Zhou, Min Zhang, Zheng Wang, Zhaoning Shi, Ruifeng Wang, Yushu Wei, Xiaolin Shang, Renjie Li, Jianwen He, Cuiyu Xie, Jin Diao, Yarui Bi, Pengpeng Humans Muscle Development Myoblasts MyoD Protein Cell Fusion Muscle, Skeletal Animals Phosphoproteins Mutation CHAMP1 is an essential regulator for human myoblast fusion and muscle development. Zhang, Haifeng Zhou, Min Zhang, Zheng Wang, Zhaoning Shi, Ruifeng Wang, Yushu Wei, Xiaolin Shang, Renjie Li, Jianwen He, Cuiyu Xie, Jin Diao, Yarui Bi, Pengpeng Humans Muscle Development Myoblasts MyoD Protein Cell Fusion Muscle, Skeletal Animals Phosphoproteins Mutation Human skeletal muscle comprises myofibers formed by fusion of thousands of myoblasts. This process depends on tightly regulated, muscle-specific fusogens, but its genetic control remains poorly understood. Here, we identify CHAMP1 (Chromosome Alignment Maintaining Phosphoprotein 1) as essential for human myoblast fusion in vitro and in vivo. Genomic and protein-interaction assays reveal a noncanonical role for CHAMP1 as a MyoD cofactor that directly activates expression of the key muscle fusogen Myomaker. As established in prior clinical reports, CHAMP1 mutations in patients cause developmental delay, hypotonia, and muscle weakness. Consistently, patient-derived cells show fusion defects that can be fully rescued by restoring Myomaker expression. Structure and function analyses identify C2H2-type zinc-finger motifs on CHAMP1 protein that are both necessary and sufficient for MyoD interaction and Myomaker expression. These findings highlight a cell-autonomous role for CHAMP1 in muscle development and disease and point to therapeutic avenues for treating CHAMP1-related muscle development defects. |
| title | CHAMP1 is an essential regulator for human myoblast fusion and muscle development. |
| topic | Humans Muscle Development Myoblasts MyoD Protein Cell Fusion Muscle, Skeletal Animals Phosphoproteins Mutation |
| url | https://pubmed.ncbi.nlm.nih.gov/41540007/ |