Muscle Stem/Progenitor Cells and Mesenchymal Stem Cells of Bone Marrow Origin for Skeletal Muscle Regeneration in Muscular Dystrophies

• Published in: Archivum Immunologiae et Therapiae Experimentalis 2018-10, Vol.66 (5), p.341-354
• Main Authors: Klimczak, Aleksandra, Kozlowska, Urszula, Kurpisz, Maciej
• Format: Article
• Language: English
• Subjects: Adaptive immunity; Animals; Anti-inflammatory agents; Biomedical and Life Sciences; Biomedicine; Bone marrow; Bone Marrow Cells - physiology; Cytokines; Growth factors; Guided Tissue Regeneration; Humans; Immune response; Immunity; Immunology; Immunomodulation; Inflammation; Inflammation - immunology; Interstitial cells; Kruppel-Like Transcription Factors - metabolism; Mesenchymal stem cells; Mesenchymal Stem Cells - physiology; Mesenchyme; Mice; Muscle Development; Muscle, Skeletal - physiology; Muscular Dystrophies - immunology; Musculoskeletal system; PAX7 Transcription Factor - metabolism; Pericytes; Pharmacology/Toxicology; Progenitor cells; Regeneration; Review; Satellite cells; Skeletal muscle; Stem cells; Stem Cells - physiology; Stromal cells
• ISSN: 0004-069X; 1661-4917
• DOI: 10.1007/s00005-018-0509-7

Muscular dystrophies represent a group of diseases which may develop in several forms, and severity of the disease is usually associated with gene mutations. In skeletal muscle regeneration and in muscular dystrophies, both innate and adaptive immune responses are involved. The regenerative potential of mesenchymal stem/stromal cells (MSCs) of bone marrow origin was confirmed by the ability to differentiate into diverse tissues and by their immunomodulatory and anti-inflammatory properties by secretion of a variety of growth factors and anti-inflammatory cytokines. Skeletal muscle comprises different types of stem/progenitor cells such as satellite cells and non-satellite stem cells including MSCs, interstitial stem cells positive for stress mediator PW1 expression and negative for PAX7 called PICs (PW1 + /PAX7 − interstitial cells), fibro/adipogenic progenitors/mesenchymal stem cells, muscle side population cells and muscle resident pericytes, and all of them actively participate in the muscle regeneration process. In this review, we present biological properties of MSCs of bone marrow origin and a heterogeneous population of muscle-resident stem/progenitor cells, their interaction with the inflammatory environment of dystrophic muscle and potential implications for cellular therapies for muscle regeneration. Subsequently, we propose—based on current research results, conclusions, and our own experience—hypothetical mechanisms for modulation of the complete muscle regeneration process to treat muscular dystrophies.