Enhanced expression of dystrophin, IGF-1, CD44 and MYH3 in plasma and skeletal muscles including diaphragm of mdx mice after oral administration of Neu REFIX beta 1,3-1,6 glucan

• Published in: Scientific reports Vol. 15; no. 1; pp. 7232 - 10
• Main Authors: Preethy, Senthilkumar, Sakamoto, Shuji, Higuchi, Takuma, Ichiyama, Koji, Yamamoto, Naoki, Ikewaki, Nobunao, Iwasaki, Masaru, Dedeepiya, Vidyasagar Devaprasad, Srinivasan, Subramaniam, Raghavan, Kadalraja, Rajmohan, Mathaiyan, Senthilkumar, Rajappa, Abraham, Samuel JK
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 28.02.2025; Nature Publishing Group; Nature Portfolio
• Subjects: 692/699; 692/699/375/374; Administration, Oral; Animals; CD44; CD44 antigen; Diaphragm; Diaphragm - drug effects; Diaphragm - metabolism; Duchenne muscular dystrophy (DMD); Duchenne's muscular dystrophy; Dystrophin; Dystrophin - blood; Dystrophin - metabolism; Dystrophy; Enzyme-linked immunosorbent assay; Genetic disorders; Glucans - administration & dosage; Glucans - pharmacology; Humanities and Social Sciences; Hyaluronan Receptors - blood; Hyaluronan Receptors - metabolism; Immunohistochemistry; Insulin-like growth factor I; Insulin-Like Growth Factor I - metabolism; Insulin-like growth factors; Male; mdx mice; Mice; Mice, Inbred mdx; multidisciplinary; Muscle, Skeletal - drug effects; Muscle, Skeletal - metabolism; Muscles; Muscular Dystrophy, Duchenne - drug therapy; Muscular Dystrophy, Duchenne - metabolism; MYH3; Myosin Heavy Chains - blood; Myosin Heavy Chains - metabolism; Oral administration; Plasma; Science; Science (multidisciplinary); Skeletal muscle; β-Glucan; MYH3; Duchenne muscular dystrophy (DMD); IGF-1; β-Glucan; mice; Dystrophin; CD44; mdx mice
• ISSN: 2045-2322; 2045-2322
• DOI: 10.1038/s41598-025-92258-4

Duchenne muscular dystrophy (DMD) is a rare genetic disease, causing muscle degeneration due to lack of dystrophin with inadequate muscle regeneration culminating in muscle dysfunction. The N-163 strain of Aureobasidium Pullulans produced Beta-1,3 − 1,6-glucan (Neu REFIX) reported to be safe with anti-inflammatory and anti-fibrotic efficacy earlier, herein we evaluated its effects on muscle regeneration in mdx mice. Forty-five mice in three groups ( n  = 15 each): Group 1 (normal), Group 2 ( mdx control), and Group 3 ( mdx fed Neu REFIX) were evaluated for 45 days. IGF-1, Dystrophin, CD44 and MYH3 in diaphragm, plasma and skeletal muscle were evaluated by ELISA and immunohistochemistry. Mean IGF-1 expression was 20.32% and 16.27% higher in plasma ( p  = 0.03) and diaphragm respectively in Neu-REFIX group. Mean dystrophin was higher in Neu-REFIX group by 70.3% and 4.7% in diaphragm and plasma respectively than control. H-score intensity of CD44 + was > 2.0 with an MYH3-positivity 20% higher in Neu-REFIX than control. Oral administration of Neu REFIX was safe. Significantly enhanced plasma IGF-1 beside increased Dystrophin, MYH3 and CD44, proving a restoration of muscle regeneration and differentiation, especially in diaphragm, makes us recommend it as a disease modifying adjuvant in both early and advanced stages of DMD.