While the expression of artificial binding proteins specifically in muscle tissue improves the dystrophic phenotype and compensates for LAMA2 deficiency in DMC 1A mouse models, it can also accentuate peripheral neuropathy and cause paralysis of the animal’s hind limbs.
A study by researchers at the Biozentrum in Switzerland shows that inducing pantissular expression of two artificial binding proteins (mini-agrin or laminin α1 and nidogen-1) in DMC 1A mouse models results in :
- almost complete restoration of normal weight, walking and muscle strength in the model mice, even in those most severely affected by the disease;
- prevent the onset of nerve damage and paralysis by restoring the disrupted myelination process in diseased mice.
