ArticleStem cell research & therapy2014
Human adipose-derived stem cell transplantation as a potential therapy for collagen VI-related congenital muscular dystrophy.
Article in Stem cell research & therapy, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed.
- Extracellular matrix in skeletal muscle injury and atrophy: mechanisms and therapeutic implications.Journal of orthopaedic translation · 2025Review
- Distinct muscle regenerative capacity of human induced pluripotent stem cell-derived mesenchymal stromal cells in Ullrich congenital muscular dystrophy model mice.Stem cell research & therapy · 2024Article
- Skeletal Muscle Regeneration in Cardiotoxin-Induced Muscle Injury Models.International journal of molecular sciences · 2022Review
- Transcription and proteome changes involved in re-innervation muscle following nerve crush in rats.BMC genomics · 2022Article
- Challenges and perspectives of tendon-derived cell therapy for tendinopathy: from bench to bedside.Stem cell research & therapy · 2022Review
- Adipose-derived stem cells decolonize skinThe Korean journal of physiology & pharmacology : official journal of the Korean Physiological Society and the Korean Society of Pharmacology · 2022Article
- Role of human adipose-derived stem cells (hADSC) on TGF-β1, type I collagen, and fibrosis degree in bladder obstruction model of Wistar rats.BMC urology · 2022Article
- Extracellular matrix: Brick and mortar in the skeletal muscle stem cell niche.Frontiers in cell and developmental biology · 2022Review
- Transcriptome analysis of collagen VI-related muscular dystrophy muscle biopsies.Annals of clinical and translational neurology · 2021Article
- Knockdown of miR-423-5p simultaneously upgrades the eNOS and VEGFa pathways in ADSCs and improves erectile function in diabetic rats.Journal of cellular and molecular medicine · 2021Article
- Collagen-VI supplementation by cell transplantation improves muscle regeneration in Ullrich congenital muscular dystrophy model mice.Stem cell research & therapy · 2021Article
- Adipose-mesenchymal stromal cells suppress experimental Sjögren syndrome by IL-33-driven expansion of ST2iScience · 2021Article
- Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice.Frontiers in cell and developmental biology · 2021Article
- Congenital muscular dystrophy-associated inflammatory chemokines provide axes for effective recruitment of therapeutic adult stem cell into muscles.Stem cell research & therapy · 2020Article
- Native extracellular matrix, synthesized ex vivo by bone marrow or adipose stromal cells, faithfully directs mesenchymal stem cell differentiation.Matrix biology plus · 2020Article
- The effect of intravenous human adipose-derived stem cells (hADSC) on transforming growth factor β1 (TGF-β1), collagen type 1, and kidney histopathological features in the unilateral ureteropelvic junction obstruction model of wistar rats.Turkish journal of urology · 2020Article
- Effect of Intracorporeal Human Adipose-Derived Stem Cells (hADSCs) on Corpora Cavernosa Transforming Growth Factor βResearch and reports in urology · 2020Article
- Basement membrane collagens and disease mechanisms.Essays in biochemistry · 2019Review
- Skeletal Muscle Regenerative Engineering.Regenerative engineering and translational medicine · 2019Article
- Regenerative Capacity of Adipose Derived Stem Cells (ADSCs), Comparison with Mesenchymal Stem Cells (MSCs).International journal of molecular sciences · 2019Review
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Authors and funding
6 authors.
Funding
Abstract
introductionCongenital muscular dystrophies (CMD) are a clinically and genetically heterogeneous group of neuromuscular disorders characterized by muscle weakness within the first two years of life. Collagen VI-related muscle disorders have recently emerged as one of the most common types of CMD. COL6 CMD is caused by deficiency and/or dysfunction of extracellular matrix (ECM) protein collagen VI. Currently, there is no specific treatment for this disabling and life-threatening disease. The primary cellular targets for collagen VI CMD therapy are fibroblasts in muscle, tendon and skin, as opposed to muscle cells for other types of muscular dystrophies. However, recent advances in stem cell research have raised the possibility that use of adult stem cells may provide dramatic new therapies for treatment of COL6 CMD.
methodsHere, we developed a procedure for isolation of human stem cells from the adipose layer of neonatal skin. The adipose-derived stem cells (ADSC) were examined for expression of ECM and related genes using gene expression array analysis. The therapeutic potential of ADSC was assessed after a single intramuscular transplantation in collagen VI-deficient mice.
resultsAnalysis of primary cultures confirmed that established ADSC represent a morphologically homogenous population with phenotypic and functional features of adult mesenchymal stem cells. A comprehensive gene expression analysis showed that ADSC express a vast array of ECM genes. Importantly, it was observed that ADSC synthesize and secrete all three collagen VI chains, suggesting suitability of ADSC for COL6 CMD treatment. Furthermore, we have found that a single intramuscular transplantation of ADSC into Col6a1-/-Rag1-/- mice under physiological and cardiotoxin-induced injury/regeneration conditions results in efficient engraftment and migration of stem cells within the skeletal muscle. Importantly, we showed that ADSC can survive long-term and continuously secrete the therapeutic collagen VI protein missing in the mutant mice.
conclusionsOverall, our findings suggest that stem cell therapy can potentially provide a new avenue for the treatment of COL6 CMD and other muscular disorders and injuries.
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