InnoBioscience is committed to Research, Discovering, and
Developing innovative therapies addressing unmet medical needs
  InnoBioscience: IB-DMD


Duchene Muscular Dystrophy and Skeletal Muscular Fibrosis

Skeletal muscular dystrophies are a group of disease that affects skeletal muscular function. Duchenne Muscular Dystrophy (DMD) is X-linked muscle wasting disease with an incidence 1 in 3500 boys. DMD is fatal by the second to third decade of age and kids are confined to use wheel chair at the age of 10-12 years old. At cellular and molecular level DMD is characterized by repeated cycles of necrosis and regeneration, progressive muscle weakness and fibrosis. The latter corresponds to the replacement of functional tissue (skeletal muscle fibers) by connective tissue or accumulation of extracellular matrix. The disease is characterized by chronic inflammation of the skeletal muscle. There no effective treatment to fight DMD or other diseases characterized by fibrotic replacement of functional tissue (lung fibrosis, hepatic fibrosis, kidney fibrosis, etc). In vitro and vivo studies suggest that the transcription factor NF-κB is recognized as an important factor in regulating the inflammatory processes and progression of the disease. In vivo IKKβ inhibition, a factor that regulates the activity of NF-κB, is an effective therapeutic approach to treat both inflammation and skeletal muscle dystrophies (DMD and others).

IB-DMD compound showed potential for reducing skeletal muscular fibrosis and muscle damage, improving muscular function, and reducing muscle tearing.

Target Indication:
Duchene Muscular Dystrophy and Fibrosis

Route of Administration:
Oral & Injectable

Mechanism of Action:

  • Inhibits NFkB and Connective Tissue Growth Factor (CTGF)
  • Lowered levels of serum Creatine Kinase
  • Reduces pro-fibrosis cytokines TGF-β1 and PAI-1 in CTGF, Fibronectine, and Collagen
  • Increases cell migration through fibrosis inhibition

Clinical Results:

  • In-vitro research had shown IB-DMD to inhibit NFkB and expression of CTGF (pro-fibrotic factor); reduce collagen, fibronectin.
  • Improves skeletal muscle strength: Increases isometric force, twitch force, and recovery score
  • Significantly improve whole animal functional capacity as determined by the ability to treated dystrophic mice to run in a treadmill.
  • Significantly decreases cumulative skeletal muscle damage, improves the architecture of dystrophic skeletal muscles, decreases tissue damage, and lowered levels of serum CK
  • Reduces Skeletal Muscle Fibrosis Proteins: fibronectin, collagen I, and collagen II
  • Reduces Pro-fibrosis Factors: TGF-β1 and CTGF
  • Reduces inflammatory Markers: macrophages, eosinophils, lymphocytes CD4+ and CD8+
  • Increases efficacy of cellular therapies
  • Extend lifespan of dystrophic mice.

Key Publication:
Skeletal Muscle (2014), 4:6 Andrographolide attenuates skeletal muscle dystrophy in mdx mice and increases efficiency of cell therapy by reducing fibrosis